Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches

Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches Wenjiao Zhu, Lei Zhang, Jing Zhang, Wei Wang, Ye Xiang, Dingxian He, and 6 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5262032/v1 This work is licensed under a CC BY 4.0 License Status: Under Review Version 1 posted 4 You are reading this latest preprint version Abstract Background Kimura disease (KD) is a rare, chronic, inflammatory disorder characterized by painless subcutaneous masses, lymphadenopathy, elevated eosinophil counts and increased serum immunoglobulin E (IgE) levels. Due to the heterogeneity and rarity, current diagnostic and treatment approaches for KD are often delayed and have limited efficacy. Methods The clinicopathological data of 51 KD patients between January 2014 and December 2023 were retrieved and stratified based on age and mass size, respectively. A case ofrecurrent KD who responded well to a low dose of mepolizumab was described. Results We found that pruritus occurred in 45.8% of patients, with higher rates in older patients ( P= 0.033). Edema was observed in 29.2%, with higher prevalence in younger patients ( P= 0.010). The mean maximum mass size was 3.1cm, varying by age group ( P= 0.005). The mean time to diagnosis was 36 months, increasing with mass size ( P= 0.024). Meanwhile, we reported that one of these 51 patients was successfully treated with mepolizumab at a dose of 100mg every 4 weeks. Conclusion We conclude that pruritus and edema are the two main clinical manifestation of KD, and the size of mass is related to the time of diagnosis. Biologic agents targeting the Th2 pathway show promise for KD treatment. Kimura disease eosinophilia pruritus mepolizumab biologics Figures Figure 1 Figure 2 Introduction Kimura disease (KD) was first described in 1937 in the Chinese literature as “eosinophilic hyperplastic lymphogranuloma” 1 and was later systematically characterized in 1948 by Kimura et al. 2 , after which it was named. KD predominantly affects young and middle-aged men between the ages of 20 and 40 3 , and is significantly more prevalent in Asia compared to Europe and America 4 , 5 . The primary manifestations of KD include slowly growing, painless subcutaneous masses, often involving the head and neck soft tissues, salivary glands or lymph nodes. These masses are frequently accompanied by elevated peripheral blood eosinophils, increased immunoglobulin E (IgE) levels, and systemic atopic symptoms such as pruritus 6 . Diagnosing KD remains challenging and requires a combination of clinical, serological, radiological, and pathological assessments. Currently, there is no consensus on optimal treatment, and existing strategies-such as surgical resection, radiotherapy, corticosteroids, and immunosuppressants-have shown limited long-term efficacy. There is growing evidence that helper T cell (Th2)-mediated eosinophilic inflammation plays a crucial role in KD 7 – 13 . Th2 cytokines, such as IL-4, IL-5, IL-9, and IL-13, have been found to be elevated in peripheral blood mononuclear cells (PBMC) and affected various tissues of patients with KD 12 , 14 . Biologic agents targeting Th2 cytokines, such as IgE, IL-5/IL-5 receptor, and IL-4/IL13 receptor alpha, are widely used in the treatment of uncontrolled, severe eosinophilic asthma 15 . However, only a few case series have demonstrated the clinical efficacy of these biologics in treating KD 16 . Given the rarity and heterogeneous clinical presentation of KD, early diagnosis and effective systemic treatment remain areas in need of significant improvement. In this study, age- and mass size-stratified analyses of 51 patients with KD were conducted to provide a comprehensive comparison of age and mass size in relation to different disease characters. Additionally, a detailed characterization of a case of KD responsive to mepolizumab was described to assit in treatment decision-making. Methods Data collection Data from 51 patients with Kimura disease (KD), pathologically confirmed for the first time at Zhongshan Hospital, Fudan University, or Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine between January 2014 and December 2023, were retrospectively collected. Pathological inclusion criteria The pathological inclusion criteria included a combination of the following features: tissue eosinophilia or eosinophilic microabscesses, florid follicular hyperplasia with prominent germinal centers, interstitial fibrosis, lymphoplasmacytic infiltrates, increased small blood vessels, and postcapillary venule proliferation. IgG4-related disease (IgG4-RD) was diagnosed according to the 2020 revised comprehensive diagnostic criteria for IgG4-RD 17 . Patient data retrieved The following parameters were retrieved for each patient: sex, age at presentation, mass distribution and morphology, associated symptoms and disorders, related laboratory abnormalities, imaging findings such as those from ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI), histopathological findings, and responses to therapy. Statistical analysis Continuous variables are expressed as medians and interquartile ranges, while categorical variables are presented as numbers and percentages. The patients were divided into groups based on age and mass size, respectively. Comparisons among the groups were performed using one-way analysis of variance (ANOVA) or Fisher’s exact probability test, except for eosinophil count and IgE level, which were compared using the Kruskal‒Wallis test due to the skewed nature of their distributions. P < 0.05 was considered to indicate statistical significance. All analyses were conducted using SPSS version 21 (IBM Corp., Armonk, N.Y., USA). Results Comprehensive analysis of clinical features A total of 51 patients (46 males and 5 females) were included in the study, with a median age of 41 years (range: 30–54). There were 6 patients under 20 years old, 18 patients aged 20–39 years, and 27 patients over 40 years old (Table 1 ). The median time to diagnosis was 34 months (range: 14–77). Patients with a mass size of < 3 cm had a median diagnosis time of 12 months, while those with mass sizes of 3-4.9 cm and ≥ 5 cm had longer times of 48 and 78 months, respectively (P = 0.024, Table 2 ). The main symptoms included subcutaneous masses with pruritus (22/48, 45.8%), skin eruptions (16/48, 33.3%), and edema (13/48, 27.1%). The frequency of pruritus increased with age (overall: 45.8%; <20 years: 0%; 20–39 years: 43.8%; ≥ 40 years: 57.7%; P = 0.033), while the prevalence of edema decreased with age (overall: 29.2%; <20 years: 83.3%; 20–39 years: 25.0%; ≥ 40 years: 19.2%; P = 0.010). Symptom frequency also varied by mass size, with skin lesions being more common in the ≥ 5 cm group ( P = 0.038) and edema more prevalent in the < 3 cm group (Table 2 ). Table 1 Clinical characteristics of Kimura’s disease by age group. Total < 20 years 20–39 years ≥ 40 years P -value Patients n = 51 n = 6 n = 18 n = 27 Sex, M:F b 46:5 5:1 17:1 24:3 0.672 Age, year 41 (30–54) 16 (13–18) 30 (27–34) 53 (47–62) <0.001 Time to diagnosis, months c 34 (14–77) 18 (8–24) 36 (22–111) 46 (15–76) 0.762 (n = 49) (n = 6) (n = 17) (n = 26) Symptoms n = 48 n = 6 n = 16 n = 26 Pruritus b 22 (45.8) 0 (0.0) 7 (43.8) 15 (57.7) 0.033 Skin lesion 16 (33.3) 0 (0.0) 6 (37.5) 10 (38.5) 0.182 Edema 14 (29.2) 5 (83.3) 4 (25.0) 5 (19.2) 0.010 Complications Atopy b 8 (16.7) 1 (16.7) 4 (25.0) 3 (11.5) 0.644 n = 48 n = 6 n = 16 n = 26 IgG4-related disease b 6 (12.2) 0 (0.0) 3 (17.6) 3 (11.5) 0.703 n = 49 n = 6 n = 17 n = 26 Thromboembolism b 4 (8.3) 0 (0.0) 4 (25.0) 0 (0.0) 0.023 n = 48 n = 6 n = 16 n = 26 Multiplicity n = 50 n = 6 n = 17 n = 27 Single b 10 (20.0) 0 (0.0) 3 (17.6) 7 (25.9) 0.426 Multiple b 40 (80.0) 6 (100.0) 14 (82.4) 20 (74.1) Laterality n = 50 n = 6 n = 17 n = 27 Unilateral b 17 (34.0) 2 (33.3) 4 (23.5) 11 (40.7) 0.483 Bilateral b 33 (66.0) 4 (66.7) 13 (76.5) 16 (59.3) Anatomical distribution Head and neck b 44 (86.3) 6 (100.0) 13 (72.2) 25 (92.6) 0.236 Parotid gland 21 (41.2) 3 (50.0) 4 (22.2) 14 (51.9) 0.148 Submandibular gland 6 (11.8) 1 (16.7) 2 (11.1) 3 (11.1) 1.000 Lacrimal gland 3 (5.9) 2 (33.3) 1 (5.6) 0 (0.0) 0.014 Lymph node 38 (74.5) 5 (83.3) 11 (61.1) 22 (81.5) 0.319 Soft tissue 20 (39.2) 3 (50.0) 6 (33.3) 11 (40.7) 0.779 Other lymph node regions Axilla b 10 (19.6) 0 (0.0) 3 (16.7) 7 (25.9) 0.425 Groin b 8 (15.7) 0 (0.0) 4 (22.2) 4 (14.8) 0.645 Cavity b 3 (5.9) 0 (0.0) 1 (5.6) 2 (7.4) 1.000 Limb b 4 (7.8) 0 (0.0) 1 (5.6) 3 (11.1) 0.785 Maximus size, cm c 3.1 (2.5–4.4) 2.5 (2.2–2.5) 3.4 (2.6–4.2) 3.5 (2.5–4.9) 0.005 (n = 49) (n = 6) (n = 16) (n = 27) Eosinophils, cells/ml d 1380 (690–3530) 2010 (1568–3535) 1530 (1108–3780) 1060 (445–3200) 0.178 (n = 47) (n = 4) (n = 16) (n = 27) Eosinophils, % d 18.6 (10.4–33.9) 23.9 (18.2–34.4) 19.4 (11.7–34.8) 13.8 (6.7–32.5) 0.460 (n = 47) (n = 4) (n = 16) (n = 27) IgE, IU/ml d 927 (267–3415) 2360 (789–8780) 2704 (841–4208) 699 (164–2640) 0.275 (n = 29) (n = 5) (n = 7) (n = 17) Initial treatment Surgery b 32 (62.7) 1 (16.7) 11 (61.1) 20 (74.1) 0.031 Steroid b 7 (13.7) 2 (33.3) 3 (16.7) 2 (7.4) 0.154 Steroid + immunosuppressants b 3 (5.9) 1 (16.7) 1 (5.6) 1 (3.7) 0.358 Surgery + Steroid b 3 (5.9) 1 (16.7) 0 (0.0) 2 (7.4) 0.218 surgery + Steroid +immunosuppressants b 2 (3.9) 0 (0.0) 1 (5.6) 1 (3.7) 0.290 Surgery + radiotherapy 3 (5.9) 1 (16.7) 1 (5.6) 1 (3.7) 0.358 Sclerotherapy b 1 (2.0) 0 (0.0) 1 (5.6) 0 (0.0) 0.471 Recurrence Yes b 31 (60.8) 5 (83.3) 7 (38.9) 19 (70.4) 0.068 No 10 (19.6) 1 (16.7) 4 (22.2) 5 (18.5) 1.000 No information b 10 (19.6) 0 (0.0) 7 (38.9) 3 (11.1) 0.045 a. Data are presented as n (%) or mean ± SD. b. Fisher exact probability test. c. one-way analysis of variance. d. Kruskal-Wallis test. Table 2 Clinical characteristics of Kimura’s disease by size group. Total < 3 cm 3-4.9cm ≥ 5 cm P -value Patients n = 49 n = 20 n = 21 n = 8 Sex, M:F b 44:5 16:4 21:0 7:1 0.101 Age, year 42 (30–54) 35 (21–45) 46 (33–53) 52 (42–60) 0.073 Time to diagnosis, months c 36 (15–78) 12 (9–24) 48 (29–108) 78 (57–204) 0.024 (n = 48) (n = 19) (n = 21) (n = 8) Symptoms n = 47 n = 19 (n = 21) n = 7 Pruritus b 21 (44.7) 6 (31.6) 8 (38.1) 7 (100.0) 0.050 Skin lesion 15 (31.9) 6 (31.6) 4 (19.0) 5 (71.4) 0.038 Oedema 14 (29.8) 10 (52.6) 1 (4.8) 3 (42.9) 0.010 Complications Atopy b 8 (17.0) 4 (21.1) 4 (19.0) 0 (0.0) 0.593 n = 47 n = 19 (n = 21) n = 7 IgG4-related disease b 5 (10.4) 0 (0.0) 3 (14.3) 2 (25.0) 0.102 n = 48 n = 19 (n = 21) n = 8 Thromboembolism b 4 (8.5) 2 (10.5) 2 (9.5) 0 (0.0) 1.000 n = 47 n = 19 (n = 21) n = 7 Multiplicity n = 48 n = 20 n = 21 n = 7 Single b 8 (16.7) 2 (10.0) 5 (23.8) 1 (14.3) 0.593 Multiple b 40 (83.3) 18 (90.0) 16 (76.2) 6 (85.7) 0.593 Laterality n = 48 n = 20 n = 21 n = 7 Unilateral b 15 (31.3) 8 (40.0) 6 (28.6) 1 (14.3) 0.439 Bilateral b 33 (68.8) 12 (60.0) 15 (71.4) 6 (85.7) 0.439 Anatomical distribution Head and neck b 42 (82.4) 18 (90.0) 17 (81.0) 7 (87.5) 0.859 Parotid gland 21 (41.2) 10 (50.0) 9 (42.9) 2 (25.0) 0.553 Submandibular gland 6 (11.8) 3 (15.0) 2 (9.5) 1 (12.5) 0.855 Lacrimal gland 3 (5.9) 3 (15.0) 0 (0.0) 0 (0.0) 0.127 Lymph node 37 (72.5) 17 (85.0) 13 (61.9) 7 (87.5) 0.222 Soft tissue 19 (37.3) 7 (35.0) 10 (47.6) 2 (25.0) 0.537 Other lymph node regions Axilla b 10 (20.4) 3 (15.0) 5 (23.8) 2 (25.0) 0.715 Groin b 8 (16.3) 2 (10.0) 3 (14.3) 3 (37.5) 0.249 Cavity b 3 (6.1) 1 (5.0) 1 (4.8) 1 (12.5) 0.555 Limb b 4 (8.2) 0 (0.0) 3 (14.3) 1 (12.5) 0.208 Maximus size, cm c 3.1 (2.5–4.4) 2.0 (1.8–2.5) 4.0 (3.1–4.2) 5.5 (5.0-6.9) <0.001 3.5 ± 1.7 2.1 ± 0.6 3.8 ± 0.7 6.3 ± 1.9 Eosinophils, cells/ml d 1360 (685–3325) 1200 (728–3373) 1410 (700–2980) 1800 (440–2810) 0.880 (n = 46) (n = 18) (n = 21) (n = 7) Eosinophils, % d 17.9 (10.2–32.8) 13.3 (9.3–39.1) 19.6 (10.8–29.1) 22.9 (6.7–32.6) 0.919 (n = 46) (n = 18) (n = 21) (n = 7) IgE, IU/ml d 789 (220–3411) 789 (281–3222) 813 (199–2500) 1882 (126–6480) 0.818 (n = 27) (n = 13) (n = 10) (n = 4) Initial treatment Surgery b 31 (63.3) 9 (45.0) 17 (81.0) 5 (62.5) 0.067 Steroid b 7 (14.3) 5 (25.0) 0 (0.0) 2 (25.0) 0.032 Surgery + Steroid b 3 (6.1) 1 (5.0) 2 (9.5) 0 (0.0) 1.000 Steroid + DMARD b 3 (6.1) 3 (15.0) 0 (0.0) 0 (0.0) 0.127 Surgery + Radiotherapy 2 (4.1) 1 (5.0) 1 (4.8) 1 (12.5) 0.555 Sclerotherapy b 1 (2.0) 0 (0.0) 1 (4.8) 0 (0.0) 1.000 Surgery + Steroid +immunosuppressants b 1 (2.0) 1 (5.0) 0 (0.0) 0 (0.0) 1.000 Recurrence Yes b 31 (63.3) 12 (60.0) 13 (61.9) 6 (75.0) 0.630 No 10 (20.4) 5 (25.0) 4 (19.0) 1 (12.5) 0.805 No information b 8 (16.3) 3 (15.0) 4 (19.0) 1 (12.5) 1.000 a. Data are presented as n (%) and median (interquartile range). b Fisher exact probability test. c one-way analysis of variance. d Kruskal-Wallis test. Comorbidities The main comorbidities were atopy (8/48, 16.7%; including atopic dermatitis in 4 patients; allergic rhinitis in 2 patients, and asthma in 3 patients), IgG4-related disease (IgG4-RD) (6/49, 12.2%), thromboembolism (4/48, 8.3%), and other conditions (Hashimoto's thyroiditis in 1 patient, nodular prurigo in 1 patient, and herpes zoster in 1 patient). There were no significant differences in the frequency of these comorbidities among the groups, except for a higher incidence of thromboembolism in the 20–39 age group. Most lesions were bilateral (33/50, 66.0%) and multiple (40/50, 80.0%). The most common anatomical sites included glands and lymph nodes in the head and neck (44/51, 86.3%), lymph nodes in other parts of the body (14/51, 27.5%) and subcutaneous tissues in the extremities (4/51, 7.8%). Notably, lacrimal gland involvement was more common in the younger group ( P = 0.014). The median maximum diameter of the masses was 3.1 cm (range: 2.5–4.4), with 2.5 cm in patients aged < 20 years, 3.4 cm in those aged 20–39, and 3.5 cm in those aged ≥ 40 years ( P = 0.005). The mean levels of peripheral eosinophils (1380 cells/ml) and IgE (927 IU/ml) were elevated in 47 and 27 patients, respectively. Bone marrow biopsies from 10 patients showed a marked eosinophil count (up to 40%) with no evidence of a myeloproliferative disorder. Additionally, 2 of the 10 patients tested positive for T-cell receptor gene rearrangement. Three specimens exhibited epithelial-like morphology along the vessel endothelium. Immunostaining showed infiltration of IgG- and IgG4-positive plasma cells in six (12%) of 41 KD cases, with two cases were diagnosed as IgG4-RD and four as “probable” IgG4-RD based on diagnostic criteria. Langerhans cell histiocytosis (CD1a+, S100+) were observed in one case. Treatment approaches Treatment options included surgical resection (32/51, 62.7%), glucocorticoids (7/51, 13.7%), glucocorticoids combined with immunosuppressants (3/51, 5.9%), sclerotherapy (1/51, 2.0%), and surgical resection combined with medications (5/51, 9.8%) or radiotherapy (3/51, 5.9%). Surgical resection was performed more frequently in the older group ( P = 0.031), while glucocorticoids treatment was less common in the 3-4.9 cm size group ( P = 0.032). Additionally, 28 of 51 patients experienced relapsed, with most undergoing surgical resection (15/28, 53.6%) as second-line treatment. There were no significant differences in sex ratio, multiplicity, laterality, serum eosinophil count, serum IgE level, or recurrence among the different age or size groups. Detailed case report of a Kimura disease patient The patient, a 34-year-old man, initially presented on May 28, 2010, with painless, solitarily, and gradually enlarging masses in the left submental region, which had been growing for three years. He also had a history of allergic rhinitis and atopic dermatitis for seven years. Laboratory tests showed a blood eosinophil count of 8500/µl (normal range < 520/µl) and a normal white blood cell count. Ultrasound examination revealed multiple lymph nodes in the neck, submental, and bilateral parotid and submandibular regions. The patient underwent surgical resection for the submental mass (maximum of 6x29 mm on ultrasound), and histopathological analysis revealed follicular hyperplasia with eosinophil infiltration. However, an ultrasound reexamination two weeks post-surgery showed recurrence at the site (maximum size of 8x21 mm). The patient did not receive adjuvant therapy following surgery. On December 19, 2017, a skin biopsy was performed due to a 12-year period of pruritus and a persistent rash on the extremities that had been poorly controlled with symptomatic treatment. The biopsy showed perivascular infiltration of lymphocytes and occasional eosinophils. On November 28, 2018, a fine-needle aspiration biopsy of a right inguinal lymph node (maximum of 40x16 mm on ultrasound), which had been detected for one year, was performed and indicated reactive hyperplasia. On December 15, 2022, a core needle biopsy of the left cervical lymph node (maximum of 21x8 mm on ultrasound) revealed a lymphoproliferative lesion with eosinophil infiltration (Fig. 1 B). Peripheral blood and bone marrow smears both showed increased eosinophil count, at 17% and 5%, respectively. The patient was diagnosed with Kimura disease, and oral prednisolone (30 mg/day) was initiated on February 11,2023. Lung function testing was performed due to new-onset shortness of breath, showing small airway dysfunction (forced expiratory flow at 50% [FEF50] 57.97%, FEF75 45.51%, and maximal mid-expiratory flow [MMEF75/25] 62.10%) with normal FEV1% predicted (83.70%, Fig. 1 C). The fractional exhaled nitric oxide (FeNO) level was 59 ppb (Fig. 1 C). Symptoms improved, and the eosinophil count decreased after prednisolone treatment but increased again during medication tapering. The patient was subsequently referred to our clinic on August 5, 2023 (x = 0 in Fig. 1 A), presenting with noticeable bilateral periauricular, submandibular and submental masses (Fig. 1 D). Laboratory tests showed a total white blood cell count of 12050/µl with eosinophilia (21.9%), serum IgE concentration of 3680 IU/mL (normal < 200 IU/mL), and serum IgG4 concentration of 31 mg/dL (normal < 200 mg/dL). A CT scan of the parotid gland revealed thickening and mild enhancement of the subcutaneous soft tissue of the bilateral preauricular, buccal and chin lymph nodes, as well as moderate enhancement of multiple enlarged lymph nodes (maximum size of 19x14 mm) in the submandibular, neck, and parotid regions (Fig. 1 E). With the patient’s consent, 100 mg of mepolizumab was administered subcutaneously every four weeks. Regression of the masses and swelling was observed (Fig. 1 E), and pruritus improved apparently at follow-up. The patient’s eosinophil count decreased, although IL-5 and IgE levels remained elevated (Fig. 1 A). The FEV1% predicted increased to 89.59%, and the FeNO levels decreased to normal (16 ppb) (Fig. 1 C). The patient continues to be actively followed up as of this report. Discussion Kimura disease (KD) is a rare, benign, chronic inflammatory condition of unknown etiology 4 . Approximately 577 cases of KD have been reported globally, with the majority occurring in Asia (530 out of 577, 92%) and a male-to-female ratio of 4.5:1 5, 16, 18–119 . The characteristics of the 51 KD patients analyzed in this study included male predominance (90%), a predilection for head and neck involvement (86.3%), pruritus as a prominent symptom (45.8%), surgery as the primary treatment (62.7%), and a high relapse rate (60.8%). These findings align with previous reports, which indicated a male-to-female ration ranging from 2:1 to 19:1, head and neck involvement rates of 71.0–91.3%, pruritus rates of 21.7–33.3%, surgery rates of 45.0–64.7%, and recurrence rates of 15–60.0% 53, 83 , 96 , 120 . Few studies have reported age- or size-specific stratification in KD. A previous study categorized 33 KD patients by mass size (< 3 cm and ≥ 3 cm) but did not identify any statistically significant differences in age, sex, clinical symptoms, lesion site, laboratory markers, or treatment regimens 121 . In this study, changes in disease patterns with age, such as the incidence of pruritus and the maximum mass size, were consistent with analyses of 238 KD patients across different regions 44 and 46 patients from another medical center in China 122 . There is significant overlap in clinical, pathological and immunohistological features between KD and IgG4-RD. A comparative study showed that 20–30% of 43 KD cases met the pathological diagnostic criteria for IgG4-RD. The equilibrium in class-switching from IgE to IgG4, induced by helper T cell (Th2) cytokines, may be a primary factor contributing to the coexistence of these two diseases 14 , 68 . The effective control of both eosinophils and IgG4-positive plasma cells under anti-IL-5 treatment in KD complicated with IgG4-RD 123 suggests a shared pathogenic basis and the therapeutic potential of targeting Th2 cytokines in both diseases. However, whether there is a potential risk of developing KD in Th2 inflammatory diseases before lymphadenopathy occurs requires further study. This case raises questions about how to consider the possibility of KD in the early stages when it only presents with symptoms of Th2 inflammatory diseases, such as skin itching. Currently, there is no standardized, evidence-based treatment for KD with proven efficacy. In the past decade, biologics targeting Th2 cytokines, such as IL-4, IL-13, IL-5, and IgE, have demonstrated efficacy in KD. An anti-IgE monoclonal antibody (mAb) (omalizumab) was initially used in a pilot study 124 , showing effectiveness in reducing mass size and eosinophil count, though IgE levels did not consistently decrease 124 , 125 . Anti-IL-4Rα mAb (dupilumab) has shown positive short-term effects in several case reports as well as in a small-scale real-word study 12 , 16 , 99 , 113 , 126 – 131 . Notably, eosinophil counts and IgE levels significantly decreased in all but one patient, who transitioned from omalizumab to dupilumab with satisfactory curative effect 131 , indicating that upstream regulator of IgE, rather than IgE itself, may play a critical role in the pathogenesis of KD. Mepolizumab, an anti-IL-5 mAb responsible for eosinophils terminal differentiation, activation, and survival 132 , has been approved for the treatment of severe eosinophilic asthma, chronic rhinosinusitis with nasal polyps(CRSwNP), eosinophilic granulomatosis with polyangiitis (EGPA), and hypereosinophilic syndrome (HES). Recent studies have reported that mepolizumab, even at one-third of the standard dose, can effectively reduce eosinophil levels and the mass size in KD patients 21 , 56 . These effects were also observed in our patient. However, the serum levels of IgE and IL-5 did not decrease, which differs from the trends with the 300mg dose of mepolizumab 123 . Although the mechanism of KD is not fully understood, the successful application of benrelizumab (anti-IL-5Rα mAb) in a patient with asthma and atopic dermatitis further supports the important of the IL-5 signaling pathway in the pathogenesis of KD 133 . The use of these biological agents targeting type 2 inflammation suggests that the key to effectively treating KD is maintaining eosinophil homeostasis in peripheral circulation and tissues by inhibiting bone marrow to blood movement with anti-IL-5 antibodies, limiting circulation to tissue transfer with anti-IL-4 antibodies, and eliminating eosinophils in blood and tissue using anti-IL-5 receptor antibodies (Fig. 2 ). Conclusion In conclusion, pruritus is a significant clinical feature of KD, and the time to diagnosis is often prolonged due to the variability in presentation and lack of specific diagnostic criteria. The complex nature of KD, with its overlapping features with IgG4-RD, highlights the role of Th2-mediated inflammation in its pathogenesis. Anti-IL-5, anti-IL-4, and anti-IL-5 receptor antibodies offer potential avenues for more targeted treatment. Future studies should focus on the mechanisms underlying KD and the role of Th2-driven inflammation, as well as the development of standardized treatment guidelines. Increased awareness and early diagnosis are critical in improving patient outcomes. Abbreviations KD Kimura disease Th2 type 2 helper T cell PBMC peripheral blood mononuclear cells CT computed tomography MRI magnetic resonance imaging IgG4-RD IgG4-related disease MAb monoclonal antibody Declarations Data availability All anonymized data in this study are available from the corresponding author upon reasonable request. Acknowledgements We are grateful to the patients for their supports and participation in this study. Funding This work was supported by the National Natural Science Foundation of China (82202054, 82270028) and Special Fund for Clinical Research, Zhongshan Hospital Affiliated to Fudan University (ZSLCYJ202330). Author information Authors and affiliations Department of Allergy, Zhongshan Hospital, Fudan University, Shanghai, 200032, China. Wenjiao Zhu, Ye Xiang, Jing Shi, Yiran Liang, Ling Ye & Meiling Jin Department of Pathology, Zhongshan Hospital, Fudan University Shanghai, 200032, China. Lei Zhang Department of Pathology, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200011, China. Jing Zhang Department of Neurosurgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200011, China. Wei Wang Huashan Rare Disease Center and Department of Neurology, Huashan Hospital, Shanghai Medical College, National Center for Neurological Disorders, Fudan University, Shanghai 200040, China. Dingxian He Department of Endocrinology and Metabolism, Peking University People's Hospital, Beijing, 100044, China. Yuanping Shi Department of Otolaryngology, Zhongshan Hospital, Fudan University, Shanghai, 200032, China. Xianhui Ning Department of Pulmonary and Critical Care Medicine, Shanghai Respiratory Research Institute, Zhongshan Hospital, Research Center of Allergy and Diseases, Fudan University, Shanghai, 200032, China. Ling Ye & Meiling Jin Contributions ZW, ZL, and ZJ designed the study and wrote the manuscript. WW, XY, HD, SJ, LY and SY contributed to data collection. ZW, NX, YL, JM performed the statistical analysis and interpretation of the results. All authors read and approved the final manuscript. Corresponding author Correspondence to Meiling Jin, Ling Ye and Xianhui Ning Ethics approval and consent to participate This study was approved by the ethics committee of Zhongshan hospital, Fudan University (B2019-020R) and Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine (SH9H-2024-T174-2). Informed consent was obtained from all patients or their guardian. 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Successful treatment of dupilumab in Kimura disease independent of IgE: A case report with literature review. Front Immunol, 2022; 13 : 1084879. Pelaia C, Paoletti G, Puggioni F, Racca F, Pelaia G, Canonica GW, et al. Interleukin-5 in the Pathophysiology of Severe Asthma. Front Physiol, 2019; 10 : 1514. Szeto VG, Chin-Yee B, Dehghani M, Rizkalla K, Licskai C, Hsia CC Successful treatment of Kimura disease with benralizumab. Ann Hematol, 2022; 101(9) : 2099-2100. Cite Share Download PDF Status: Under Review Version 1 posted Reviewers agreed at journal 18 Jan, 2025 Reviewers invited by journal 25 Oct, 2024 Editor assigned by journal 22 Oct, 2024 First submitted to journal 19 Oct, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-5262032","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":370515040,"identity":"8fc60faf-c416-4b11-8784-1aed79921339","order_by":0,"name":"Wenjiao Zhu","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Wenjiao","middleName":"","lastName":"Zhu","suffix":""},{"id":370515041,"identity":"f0f83d60-e351-4673-a61b-5c4a29f04006","order_by":1,"name":"Lei Zhang","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Lei","middleName":"","lastName":"Zhang","suffix":""},{"id":370515042,"identity":"51100651-fb2e-4fd2-9695-1ad8bd57d6ad","order_by":2,"name":"Jing Zhang","email":"","orcid":"","institution":"Shanghai Jiao Tong University School of Medicine Affiliated Ninth People's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Jing","middleName":"","lastName":"Zhang","suffix":""},{"id":370515043,"identity":"41efdd81-3b9a-4660-9a2e-62cff5d129af","order_by":3,"name":"Wei Wang","email":"","orcid":"","institution":"Shanghai Jiao Tong University School of Medicine Affiliated Ninth People's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Wei","middleName":"","lastName":"Wang","suffix":""},{"id":370515044,"identity":"1ca5635a-270a-4f37-a24f-05428b50035a","order_by":4,"name":"Ye Xiang","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Ye","middleName":"","lastName":"Xiang","suffix":""},{"id":370515045,"identity":"4e376cfd-ad78-4b80-96c1-d8c14c9bd8d0","order_by":5,"name":"Dingxian He","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Dingxian","middleName":"","lastName":"He","suffix":""},{"id":370515046,"identity":"8e0d977d-62c1-4436-97bc-db526aa79540","order_by":6,"name":"Jing Shi","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Jing","middleName":"","lastName":"Shi","suffix":""},{"id":370515047,"identity":"571f4d75-8976-411e-82a2-b40670b4f7c6","order_by":7,"name":"Yiran Liang","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Yiran","middleName":"","lastName":"Liang","suffix":""},{"id":370515048,"identity":"d7d52bee-943f-445d-856e-ff27565025be","order_by":8,"name":"Yuanping Shi","email":"","orcid":"","institution":"Peking University People's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Yuanping","middleName":"","lastName":"Shi","suffix":""},{"id":370515049,"identity":"5f68a31c-57ec-4dbf-8f8f-5bddf414c148","order_by":9,"name":"Xianhui Ning","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Xianhui","middleName":"","lastName":"Ning","suffix":""},{"id":370515050,"identity":"457019be-deaf-48eb-a43c-ed59f121a1df","order_by":10,"name":"Ling Ye","email":"","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Ling","middleName":"","lastName":"Ye","suffix":""},{"id":370515051,"identity":"6b873423-f837-424c-9ef4-4641a9e5cbc9","order_by":11,"name":"Meiling Jin","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAsElEQVRIiWNgGAWjYDACdoaEAwwGEnL8zMyHHxCnhRmkpcDCWLKdLc2AWC1A8KEiccN5HgUJonToNjM8PFxgIGFsfJiHwYChxiaaoBazwwwJh2cA/WJ2mPfAA4ZjabkNRGnhAdpidpgvwYCx4TDxWhI3NwNJ0rRsYCZVi7HEYWAgJxDll+M9yZ95/tTJ8fcfPvzgQ40NYS0MDDwJCHYCLkWogP0AcepGwSgYBaNg5AIAo+s8ricQybUAAAAASUVORK5CYII=","orcid":"","institution":"Zhongshan Hospital Fudan University","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Meiling","middleName":"","lastName":"Jin","suffix":""}],"badges":[],"createdAt":"2024-10-14 14:49:12","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-5262032/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-5262032/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":67765455,"identity":"d9d117f1-13bd-4594-a7a2-829993053efb","added_by":"auto","created_at":"2024-10-29 13:06:35","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":683567,"visible":true,"origin":"","legend":"\u003cp\u003eClinical and laboratory parameters of KD051. A, Clinical course before and after mepolizumab treatment. The time of treatment initiation with 100mg mepolizumab every 4 weeks is marked as 0 (August 10, 2023). The graph shows values of the following parameters over the entire course: blood eosinophil count (x100 μL; red line), eosinophil proportion (%; prune line), serum IgE (g/dL; blue line), serum IL-5 (pg/mL; green line), prednisone treatment dosage (mg/day; grey area), and mepolizumab treatment (100 mg every 4 weeks; black dashed line). B, Histological images from left cervical lymph node needle biopsy showing a dense cell infiltrate consisting mainly of lymphocytes with some eosinophils. The lower image is a magnified view of the highlighted black box in the upper image. C, The bronchodilator response in FEV1% predicted and fractional exhaled nitric oxide (FeNO) values before and after 9 months of mepolizumab treatment. D, Clinical presentation of Kimura disease at baseline (left panel) and response after 9 months of mepolizumab treatment (right panel). E, Axial contrast-enhanced computed tomography (CT) images taken before mepolizumab treatment showing two well-defined masses located in the bilateral preauricular regions (left panel). The lesions show a homogeneous moderate enhancement (right panel).\u003c/p\u003e","description":"","filename":"Figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-5262032/v1/f932211d9871b679eda9b9dd.png"},{"id":67765454,"identity":"4d413c1d-01a7-40b5-94a6-a753aa3ae692","added_by":"auto","created_at":"2024-10-29 13:06:35","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1117464,"visible":true,"origin":"","legend":"\u003cp\u003eOverview of biologics currently used for treating Kimura disease. Omalizumab is an anti-IgE monoclonal antibody. Dupilumab binds to the alpha subunit of the interleukin-4 receptor. Mepolizumab is a recombinant anti-IL5 humanized monoclonal antibody. Benralizumab binds to the α chain of IL-5 receptor α. Abbreviations: DC, dendritic cell; IgE, immunoglobulin E; IL, interleukin; ILC2, innate lymphoid cell type‐2; EOS, eosinophil; MC, mast cell; ASC, antibody-secreting cell.\u003c/p\u003e","description":"","filename":"Figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-5262032/v1/2785bf483dd65ae9968f9171.png"},{"id":67766351,"identity":"e36c0662-79b5-4c1e-a684-82c4ce51d662","added_by":"auto","created_at":"2024-10-29 13:14:36","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3131220,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-5262032/v1/d570c3ff-3de3-4f6a-9381-8c1901668b34.pdf"}],"financialInterests":"","formattedTitle":"Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches","fulltext":[{"header":"Introduction","content":"\u003cp\u003eKimura disease (KD) was first described in 1937 in the Chinese literature as \u0026ldquo;eosinophilic hyperplastic lymphogranuloma\u0026rdquo;\u003csup\u003e\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u003c/sup\u003e and was later systematically characterized in 1948 by Kimura et al.\u003csup\u003e\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u003c/sup\u003e, after which it was named. KD predominantly affects young and middle-aged men between the ages of 20 and 40\u003csup\u003e3\u003c/sup\u003e, and is significantly more prevalent in Asia compared to Europe and America\u003csup\u003e\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e\u003c/sup\u003e. The primary manifestations of KD include slowly growing, painless subcutaneous masses, often involving the head and neck soft tissues, salivary glands or lymph nodes. These masses are frequently accompanied by elevated peripheral blood eosinophils, increased immunoglobulin E (IgE) levels, and systemic atopic symptoms such as pruritus\u003csup\u003e\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u003c/sup\u003e. Diagnosing KD remains challenging and requires a combination of clinical, serological, radiological, and pathological assessments. Currently, there is no consensus on optimal treatment, and existing strategies-such as surgical resection, radiotherapy, corticosteroids, and immunosuppressants-have shown limited long-term efficacy.\u003c/p\u003e \u003cp\u003eThere is growing evidence that helper T cell (Th2)-mediated eosinophilic inflammation plays a crucial role in KD\u003csup\u003e\u003cspan additionalcitationids=\"CR8 CR9 CR10 CR11 CR12\" citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e\u003c/sup\u003e. Th2 cytokines, such as IL-4, IL-5, IL-9, and IL-13, have been found to be elevated in peripheral blood mononuclear cells (PBMC) and affected various tissues of patients with KD\u003csup\u003e\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e\u003c/sup\u003e. Biologic agents targeting Th2 cytokines, such as IgE, IL-5/IL-5 receptor, and IL-4/IL13 receptor alpha, are widely used in the treatment of uncontrolled, severe eosinophilic asthma\u003csup\u003e\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u003c/sup\u003e. However, only a few case series have demonstrated the clinical efficacy of these biologics in treating KD\u003csup\u003e\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e\u003c/sup\u003e. Given the rarity and heterogeneous clinical presentation of KD, early diagnosis and effective systemic treatment remain areas in need of significant improvement. In this study, age- and mass size-stratified analyses of 51 patients with KD were conducted to provide a comprehensive comparison of age and mass size in relation to different disease characters. Additionally, a detailed characterization of a case of KD responsive to mepolizumab was described to assit in treatment decision-making.\u003c/p\u003e"},{"header":"Methods","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eData collection\u003c/h2\u003e \u003cp\u003eData from 51 patients with Kimura disease (KD), pathologically confirmed for the first time at Zhongshan Hospital, Fudan University, or Shanghai Ninth People\u0026rsquo;s Hospital, Shanghai Jiao Tong University School of Medicine between January 2014 and December 2023, were retrospectively collected.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003ePathological inclusion criteria\u003c/h3\u003e\n\u003cp\u003eThe pathological inclusion criteria included a combination of the following features: tissue eosinophilia or eosinophilic microabscesses, florid follicular hyperplasia with prominent germinal centers, interstitial fibrosis, lymphoplasmacytic infiltrates, increased small blood vessels, and postcapillary venule proliferation. IgG4-related disease (IgG4-RD) was diagnosed according to the 2020 revised comprehensive diagnostic criteria for IgG4-RD\u003csup\u003e\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e\u003c/sup\u003e.\u003c/p\u003e\n\u003ch3\u003ePatient data retrieved\u003c/h3\u003e\n\u003cp\u003eThe following parameters were retrieved for each patient: sex, age at presentation, mass distribution and morphology, associated symptoms and disorders, related laboratory abnormalities, imaging findings such as those from ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI), histopathological findings, and responses to therapy.\u003c/p\u003e \u003cdiv id=\"Sec6\" class=\"Section2\"\u003e \u003ch2\u003eStatistical analysis\u003c/h2\u003e \u003cp\u003eContinuous variables are expressed as medians and interquartile ranges, while categorical variables are presented as numbers and percentages. The patients were divided into groups based on age and mass size, respectively. Comparisons among the groups were performed using one-way analysis of variance (ANOVA) or Fisher\u0026rsquo;s exact probability test, except for eosinophil count and IgE level, which were compared using the Kruskal‒Wallis test due to the skewed nature of their distributions. \u003cem\u003eP\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.05 was considered to indicate statistical significance. All analyses were conducted using SPSS version 21 (IBM Corp., Armonk, N.Y., USA).\u003c/p\u003e \u003c/div\u003e"},{"header":"Results","content":"\u003cdiv id=\"Sec8\" class=\"Section2\"\u003e \u003ch2\u003eComprehensive analysis of clinical features\u003c/h2\u003e \u003cp\u003eA total of 51 patients (46 males and 5 females) were included in the study, with a median age of 41 years (range: 30\u0026ndash;54). There were 6 patients under 20 years old, 18 patients aged 20\u0026ndash;39 years, and 27 patients over 40 years old (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). The median time to diagnosis was 34 months (range: 14\u0026ndash;77). Patients with a mass size of \u0026lt;\u0026thinsp;3 cm had a median diagnosis time of 12 months, while those with mass sizes of 3-4.9 cm and \u0026ge;\u0026thinsp;5 cm had longer times of 48 and 78 months, respectively (P\u0026thinsp;=\u0026thinsp;0.024, Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). The main symptoms included subcutaneous masses with pruritus (22/48, 45.8%), skin eruptions (16/48, 33.3%), and edema (13/48, 27.1%). The frequency of pruritus increased with age (overall: 45.8%; \u0026lt;20 years: 0%; 20\u0026ndash;39 years: 43.8%; \u0026ge; 40 years: 57.7%; \u003cem\u003eP\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.033), while the prevalence of edema decreased with age (overall: 29.2%; \u0026lt;20 years: 83.3%; 20\u0026ndash;39 years: 25.0%; \u0026ge; 40 years: 19.2%; \u003cem\u003eP\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.010). Symptom frequency also varied by mass size, with skin lesions being more common in the \u0026ge;\u0026thinsp;5 cm group (\u003cem\u003eP\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.038) and edema more prevalent in the \u0026lt;\u0026thinsp;3 cm group (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eClinical characteristics of Kimura\u0026rsquo;s disease by age group.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"6\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eTotal\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;20 years\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003e20\u0026ndash;39 years\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003e\u0026ge;\u0026thinsp;40 years\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e\u003cem\u003eP\u003c/em\u003e-value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;51\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u003cem\u003en\u003c/em\u003e\u0026thinsp;=\u0026thinsp;6\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cem\u003en\u003c/em\u003e\u0026thinsp;=\u0026thinsp;18\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003e\u003cem\u003en\u003c/em\u003e\u0026thinsp;=\u0026thinsp;27\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSex, M:F\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e46:5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5:1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e17:1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e24:3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.672\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAge, year\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e41 (30\u0026ndash;54)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e16 (13\u0026ndash;18)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e30 (27\u0026ndash;34)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e53 (47\u0026ndash;62)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e\u0026lt;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eTime to diagnosis, months\u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e34 (14\u0026ndash;77)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e18 (8\u0026ndash;24)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e36 (22\u0026ndash;111)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e46 (15\u0026ndash;76)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.762\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;49)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;17)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;26)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSymptoms\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;48\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;16\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;26\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePruritus\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e22 (45.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e7 (43.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e15 (57.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.033\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSkin lesion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e16 (33.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e6 (37.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e10 (38.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.182\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEdema\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e14 (29.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 (83.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e5 (19.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.010\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eComplications\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eAtopy\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3 (11.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.644\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;48\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;16\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;26\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eIgG4-related disease\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (12.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (17.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3 (11.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.703\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;49\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;17\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;26\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eThromboembolism\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (8.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.023\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;48\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;16\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;26\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMultiplicity\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;17\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;27\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSingle\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (20.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (17.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e7 (25.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.426\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMultiple\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e40 (80.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6 (100.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e14 (82.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e20 (74.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLaterality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;17\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;27\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eUnilateral\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e17 (34.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2 (33.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (23.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e11 (40.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.483\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBilateral\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e33 (66.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4 (66.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e13 (76.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e16 (59.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eAnatomical distribution\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eHead and neck\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e44 (86.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6 (100.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e13 (72.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e25 (92.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.236\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eParotid gland\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e21 (41.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (50.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (22.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e14 (51.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.148\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSubmandibular gland\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (11.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2 (11.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3 (11.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLacrimal gland\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (5.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2 (33.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (5.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.014\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLymph node\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e38 (74.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 (83.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e11 (61.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e22 (81.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.319\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSoft tissue\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e20 (39.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (50.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e6 (33.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e11 (40.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.779\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eOther lymph node regions\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAxilla\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (19.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e7 (25.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.425\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGroin\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (15.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (22.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e4 (14.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.645\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCavity\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (5.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (5.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (7.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLimb\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (7.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (5.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3 (11.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.785\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eMaximus size, cm\u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3.1 (2.5\u0026ndash;4.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2.5 (2.2\u0026ndash;2.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3.4 (2.6\u0026ndash;4.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3.5 (2.5\u0026ndash;4.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.005\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;49)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;16)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;27)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eEosinophils, cells/ml\u003csup\u003ed\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1380 (690\u0026ndash;3530)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2010 (1568\u0026ndash;3535)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1530 (1108\u0026ndash;3780)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1060 (445\u0026ndash;3200)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.178\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;47)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;16)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;27)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eEosinophils, %\u003csup\u003ed\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e18.6 (10.4\u0026ndash;33.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e23.9 (18.2\u0026ndash;34.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e19.4 (11.7\u0026ndash;34.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e13.8 (6.7\u0026ndash;32.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.460\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;47)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;16)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;27)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eIgE, IU/ml\u003csup\u003ed\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e927 (267\u0026ndash;3415)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2360 (789\u0026ndash;8780)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2704 (841\u0026ndash;4208)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e699 (164\u0026ndash;2640)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.275\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;29)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;17)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eInitial treatment\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgery\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e32 (62.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e11 (61.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e20 (74.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.031\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSteroid\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7 (13.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2 (33.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (7.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.154\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSteroid\u0026thinsp;+\u0026thinsp;immunosuppressants\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (5.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (5.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (3.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.358\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgery\u0026thinsp;+\u0026thinsp;Steroid\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (5.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (7.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.218\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003esurgery\u0026thinsp;+\u0026thinsp;Steroid\u003c/p\u003e \u003cp\u003e+immunosuppressants\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (3.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (5.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (3.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.290\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgery\u0026thinsp;+\u0026thinsp;radiotherapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (5.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (5.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (3.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.358\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSclerotherapy\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (2.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (5.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.471\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eRecurrence\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eYes\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e31 (60.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 (83.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e7 (38.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e19 (70.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.068\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNo\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (19.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (22.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e5 (18.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNo information\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (19.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e7 (38.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3 (11.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.045\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003ea. Data are presented as n (%) or mean\u0026thinsp;\u0026plusmn;\u0026thinsp;SD.\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003eb. Fisher exact probability test.\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003ec. one-way analysis of variance.\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003ed. Kruskal-Wallis test.\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eClinical characteristics of Kimura\u0026rsquo;s disease by size group.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"6\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eTotal\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;3 cm\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3-4.9cm\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003e\u0026ge;\u0026thinsp;5 cm\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003e\u003cem\u003eP\u003c/em\u003e-value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;49\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u003cem\u003en\u003c/em\u003e\u0026thinsp;=\u0026thinsp;20\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cem\u003en\u003c/em\u003e\u0026thinsp;=\u0026thinsp;21\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003e\u003cem\u003en\u003c/em\u003e\u0026thinsp;=\u0026thinsp;8\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSex, M:F\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e44:5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e16:4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e21:0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e7:1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.101\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAge, year\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e42 (30\u0026ndash;54)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e35 (21\u0026ndash;45)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e46 (33\u0026ndash;53)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e52 (42\u0026ndash;60)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.073\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eTime to diagnosis, months\u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e36 (15\u0026ndash;78)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e12 (9\u0026ndash;24)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e48 (29\u0026ndash;108)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e78 (57\u0026ndash;204)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.024\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;48)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;19)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSymptoms\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;47\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePruritus\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e21 (44.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6 (31.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e8 (38.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e7 (100.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.050\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSkin lesion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e15 (31.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6 (31.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (19.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e5 (71.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.038\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eOedema\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e14 (29.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e10 (52.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (4.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3 (42.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.010\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eComplications\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eAtopy\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (17.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4 (21.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (19.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.593\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;47\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eIgG4-related disease\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e5 (10.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (14.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.102\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;48\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eThromboembolism\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (8.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2 (10.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2 (9.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;47\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMultiplicity\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;48\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;20\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;21\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSingle\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (16.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2 (10.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e5 (23.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (14.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.593\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMultiple\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e40 (83.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e18 (90.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e16 (76.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e6 (85.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.593\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLaterality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;48\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;20\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;21\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eUnilateral\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e15 (31.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e8 (40.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e6 (28.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (14.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.439\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBilateral\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e33 (68.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e12 (60.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e15 (71.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e6 (85.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.439\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eAnatomical distribution\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eHead and neck\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e42 (82.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e18 (90.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e17 (81.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e7 (87.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.859\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eParotid gland\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e21 (41.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e10 (50.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e9 (42.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.553\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSubmandibular gland\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (11.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (15.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2 (9.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (12.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.855\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLacrimal gland\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (5.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (15.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.127\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLymph node\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e37 (72.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e17 (85.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e13 (61.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e7 (87.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.222\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSoft tissue\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e19 (37.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e7 (35.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10 (47.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.537\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eOther lymph node regions\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAxilla\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (20.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (15.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e5 (23.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.715\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGroin\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (16.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2 (10.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (14.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3 (37.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.249\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCavity\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (6.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (5.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (4.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (12.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.555\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLimb\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (8.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (14.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (12.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.208\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eMaximus size, cm\u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3.1 (2.5\u0026ndash;4.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2.0 (1.8\u0026ndash;2.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4.0 (3.1\u0026ndash;4.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e5.5 (5.0-6.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e\u0026lt;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3.5\u0026thinsp;\u0026plusmn;\u0026thinsp;1.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2.1\u0026thinsp;\u0026plusmn;\u0026thinsp;0.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3.8\u0026thinsp;\u0026plusmn;\u0026thinsp;0.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e6.3\u0026thinsp;\u0026plusmn;\u0026thinsp;1.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eEosinophils, cells/ml\u003csup\u003ed\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1360 (685\u0026ndash;3325)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1200 (728\u0026ndash;3373)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1410 (700\u0026ndash;2980)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1800 (440\u0026ndash;2810)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.880\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;46)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;18)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eEosinophils, %\u003csup\u003ed\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e17.9 (10.2\u0026ndash;32.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e13.3 (9.3\u0026ndash;39.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e19.6 (10.8\u0026ndash;29.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e22.9 (6.7\u0026ndash;32.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.919\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;46)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;18)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eIgE, IU/ml\u003csup\u003ed\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e789 (220\u0026ndash;3411)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e789 (281\u0026ndash;3222)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e813 (199\u0026ndash;2500)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1882 (126\u0026ndash;6480)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.818\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;27)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;13)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;10)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e(n\u0026thinsp;=\u0026thinsp;4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eInitial treatment\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgery\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e31 (63.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e9 (45.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e17 (81.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e5 (62.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.067\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSteroid\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7 (14.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.032\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgery\u0026thinsp;+\u0026thinsp;Steroid\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (6.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (5.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2 (9.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSteroid\u0026thinsp;+\u0026thinsp;DMARD\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (6.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (15.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.127\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgery\u0026thinsp;+\u0026thinsp;Radiotherapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (4.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (5.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (4.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (12.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.555\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSclerotherapy\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (2.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (4.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgery\u0026thinsp;+\u0026thinsp;Steroid\u003c/p\u003e \u003cp\u003e+immunosuppressants\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (2.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (5.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"6\" nameend=\"c6\" namest=\"c1\"\u003e \u003cp\u003eRecurrence\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eYes\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e31 (63.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e12 (60.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e13 (61.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e6 (75.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.630\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNo\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (20.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 (25.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (19.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (12.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.805\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNo information\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (16.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (15.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (19.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e1 (12.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003ea. Data are presented as n (%) and median (interquartile range).\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003eb Fisher exact probability test.\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003ec one-way analysis of variance.\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003ed Kruskal-Wallis test.\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eComorbidities\u003c/h3\u003e\n\u003cp\u003eThe main comorbidities were atopy (8/48, 16.7%; including atopic dermatitis in 4 patients; allergic rhinitis in 2 patients, and asthma in 3 patients), IgG4-related disease (IgG4-RD) (6/49, 12.2%), thromboembolism (4/48, 8.3%), and other conditions (Hashimoto's thyroiditis in 1 patient, nodular prurigo in 1 patient, and herpes zoster in 1 patient). There were no significant differences in the frequency of these comorbidities among the groups, except for a higher incidence of thromboembolism in the 20\u0026ndash;39 age group. Most lesions were bilateral (33/50, 66.0%) and multiple (40/50, 80.0%). The most common anatomical sites included glands and lymph nodes in the head and neck (44/51, 86.3%), lymph nodes in other parts of the body (14/51, 27.5%) and subcutaneous tissues in the extremities (4/51, 7.8%). Notably, lacrimal gland involvement was more common in the younger group (\u003cem\u003eP\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.014). The median maximum diameter of the masses was 3.1 cm (range: 2.5\u0026ndash;4.4), with 2.5 cm in patients aged\u0026thinsp;\u0026lt;\u0026thinsp;20 years, 3.4 cm in those aged 20\u0026ndash;39, and 3.5 cm in those aged\u0026thinsp;\u0026ge;\u0026thinsp;40 years (\u003cem\u003eP\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.005).\u003c/p\u003e \u003cp\u003eThe mean levels of peripheral eosinophils (1380 cells/ml) and IgE (927 IU/ml) were elevated in 47 and 27 patients, respectively. Bone marrow biopsies from 10 patients showed a marked eosinophil count (up to 40%) with no evidence of a myeloproliferative disorder. Additionally, 2 of the 10 patients tested positive for T-cell receptor gene rearrangement. Three specimens exhibited epithelial-like morphology along the vessel endothelium. Immunostaining showed infiltration of IgG- and IgG4-positive plasma cells in six (12%) of 41 KD cases, with two cases were diagnosed as IgG4-RD and four as \u0026ldquo;probable\u0026rdquo; IgG4-RD based on diagnostic criteria. Langerhans cell histiocytosis (CD1a+, S100+) were observed in one case.\u003c/p\u003e\n\u003ch3\u003eTreatment approaches\u003c/h3\u003e\n\u003cp\u003eTreatment options included surgical resection (32/51, 62.7%), glucocorticoids (7/51, 13.7%), glucocorticoids combined with immunosuppressants (3/51, 5.9%), sclerotherapy (1/51, 2.0%), and surgical resection combined with medications (5/51, 9.8%) or radiotherapy (3/51, 5.9%). Surgical resection was performed more frequently in the older group (\u003cem\u003eP\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.031), while glucocorticoids treatment was less common in the 3-4.9 cm size group (\u003cem\u003eP\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.032). Additionally, 28 of 51 patients experienced relapsed, with most undergoing surgical resection (15/28, 53.6%) as second-line treatment. There were no significant differences in sex ratio, multiplicity, laterality, serum eosinophil count, serum IgE level, or recurrence among the different age or size groups.\u003c/p\u003e \u003cdiv id=\"Sec11\" class=\"Section2\"\u003e \u003ch2\u003eDetailed case report of a Kimura disease patient\u003c/h2\u003e \u003cp\u003eThe patient, a 34-year-old man, initially presented on May 28, 2010, with painless, solitarily, and gradually enlarging masses in the left submental region, which had been growing for three years. He also had a history of allergic rhinitis and atopic dermatitis for seven years. Laboratory tests showed a blood eosinophil count of 8500/\u0026micro;l (normal range\u0026thinsp;\u0026lt;\u0026thinsp;520/\u0026micro;l) and a normal white blood cell count. Ultrasound examination revealed multiple lymph nodes in the neck, submental, and bilateral parotid and submandibular regions. The patient underwent surgical resection for the submental mass (maximum of 6x29 mm on ultrasound), and histopathological analysis revealed follicular hyperplasia with eosinophil infiltration. However, an ultrasound reexamination two weeks post-surgery showed recurrence at the site (maximum size of 8x21 mm). The patient did not receive adjuvant therapy following surgery.\u003c/p\u003e \u003cp\u003eOn December 19, 2017, a skin biopsy was performed due to a 12-year period of pruritus and a persistent rash on the extremities that had been poorly controlled with symptomatic treatment. The biopsy showed perivascular infiltration of lymphocytes and occasional eosinophils. On November 28, 2018, a fine-needle aspiration biopsy of a right inguinal lymph node (maximum of 40x16 mm on ultrasound), which had been detected for one year, was performed and indicated reactive hyperplasia.\u003c/p\u003e \u003cp\u003eOn December 15, 2022, a core needle biopsy of the left cervical lymph node (maximum of 21x8 mm on ultrasound) revealed a lymphoproliferative lesion with eosinophil infiltration (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eB). Peripheral blood and bone marrow smears both showed increased eosinophil count, at 17% and 5%, respectively. The patient was diagnosed with Kimura disease, and oral prednisolone (30 mg/day) was initiated on February 11,2023. Lung function testing was performed due to new-onset shortness of breath, showing small airway dysfunction (forced expiratory flow at 50% [FEF50] 57.97%, FEF75 45.51%, and maximal mid-expiratory flow [MMEF75/25] 62.10%) with normal FEV1% predicted (83.70%, Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eC). The fractional exhaled nitric oxide (FeNO) level was 59 ppb (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eC). Symptoms improved, and the eosinophil count decreased after prednisolone treatment but increased again during medication tapering.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe patient was subsequently referred to our clinic on August 5, 2023 (x\u0026thinsp;=\u0026thinsp;0 in Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eA), presenting with noticeable bilateral periauricular, submandibular and submental masses (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eD). Laboratory tests showed a total white blood cell count of 12050/\u0026micro;l with eosinophilia (21.9%), serum IgE concentration of 3680 IU/mL (normal\u0026thinsp;\u0026lt;\u0026thinsp;200 IU/mL), and serum IgG4 concentration of 31 mg/dL (normal\u0026thinsp;\u0026lt;\u0026thinsp;200 mg/dL). A CT scan of the parotid gland revealed thickening and mild enhancement of the subcutaneous soft tissue of the bilateral preauricular, buccal and chin lymph nodes, as well as moderate enhancement of multiple enlarged lymph nodes (maximum size of 19x14 mm) in the submandibular, neck, and parotid regions (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eE). With the patient\u0026rsquo;s consent, 100 mg of mepolizumab was administered subcutaneously every four weeks. Regression of the masses and swelling was observed (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eE), and pruritus improved apparently at follow-up. The patient\u0026rsquo;s eosinophil count decreased, although IL-5 and IgE levels remained elevated (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eA). The FEV1% predicted increased to 89.59%, and the FeNO levels decreased to normal (16 ppb) (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eC). The patient continues to be actively followed up as of this report.\u003c/p\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003eKimura disease (KD) is a rare, benign, chronic inflammatory condition of unknown etiology\u003csup\u003e\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e\u003c/sup\u003e. Approximately 577 cases of KD have been reported globally, with the majority occurring in Asia (530 out of 577, 92%) and a male-to-female ratio of 4.5:1\u003csup\u003e5, 16, 18\u0026ndash;119\u003c/sup\u003e. The characteristics of the 51 KD patients analyzed in this study included male predominance (90%), a predilection for head and neck involvement (86.3%), pruritus as a prominent symptom (45.8%), surgery as the primary treatment (62.7%), and a high relapse rate (60.8%). These findings align with previous reports, which indicated a male-to-female ration ranging from 2:1 to 19:1, head and neck involvement rates of 71.0\u0026ndash;91.3%, pruritus rates of 21.7\u0026ndash;33.3%, surgery rates of 45.0\u0026ndash;64.7%, and recurrence rates of 15\u0026ndash;60.0%\u003csup\u003e53, \u003cspan citationid=\"CR83\" class=\"CitationRef\"\u003e83\u003c/span\u003e, \u003cspan citationid=\"CR96\" class=\"CitationRef\"\u003e96\u003c/span\u003e, \u003cspan citationid=\"CR120\" class=\"CitationRef\"\u003e120\u003c/span\u003e\u003c/sup\u003e.\u003c/p\u003e \u003cp\u003eFew studies have reported age- or size-specific stratification in KD. A previous study categorized 33 KD patients by mass size (\u0026lt;\u0026thinsp;3 cm and \u0026ge;\u0026thinsp;3 cm) but did not identify any statistically significant differences in age, sex, clinical symptoms, lesion site, laboratory markers, or treatment regimens\u003csup\u003e\u003cspan citationid=\"CR121\" class=\"CitationRef\"\u003e121\u003c/span\u003e\u003c/sup\u003e. In this study, changes in disease patterns with age, such as the incidence of pruritus and the maximum mass size, were consistent with analyses of 238 KD patients across different regions\u003csup\u003e\u003cspan citationid=\"CR44\" class=\"CitationRef\"\u003e44\u003c/span\u003e\u003c/sup\u003e and 46 patients from another medical center in China\u003csup\u003e\u003cspan citationid=\"CR122\" class=\"CitationRef\"\u003e122\u003c/span\u003e\u003c/sup\u003e.\u003c/p\u003e \u003cp\u003eThere is significant overlap in clinical, pathological and immunohistological features between KD and IgG4-RD. A comparative study showed that 20\u0026ndash;30% of 43 KD cases met the pathological diagnostic criteria for IgG4-RD. The equilibrium in class-switching from IgE to IgG4, induced by helper T cell (Th2) cytokines, may be a primary factor contributing to the coexistence of these two diseases\u003csup\u003e\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e, \u003cspan citationid=\"CR68\" class=\"CitationRef\"\u003e68\u003c/span\u003e\u003c/sup\u003e. The effective control of both eosinophils and IgG4-positive plasma cells under anti-IL-5 treatment in KD complicated with IgG4-RD\u003csup\u003e\u003cspan citationid=\"CR123\" class=\"CitationRef\"\u003e123\u003c/span\u003e\u003c/sup\u003e suggests a shared pathogenic basis and the therapeutic potential of targeting Th2 cytokines in both diseases. However, whether there is a potential risk of developing KD in Th2 inflammatory diseases before lymphadenopathy occurs requires further study. This case raises questions about how to consider the possibility of KD in the early stages when it only presents with symptoms of Th2 inflammatory diseases, such as skin itching.\u003c/p\u003e \u003cp\u003eCurrently, there is no standardized, evidence-based treatment for KD with proven efficacy. In the past decade, biologics targeting Th2 cytokines, such as IL-4, IL-13, IL-5, and IgE, have demonstrated efficacy in KD. An anti-IgE monoclonal antibody (mAb) (omalizumab) was initially used in a pilot study\u003csup\u003e\u003cspan citationid=\"CR124\" class=\"CitationRef\"\u003e124\u003c/span\u003e\u003c/sup\u003e, showing effectiveness in reducing mass size and eosinophil count, though IgE levels did not consistently decrease\u003csup\u003e\u003cspan citationid=\"CR124\" class=\"CitationRef\"\u003e124\u003c/span\u003e, \u003cspan citationid=\"CR125\" class=\"CitationRef\"\u003e125\u003c/span\u003e\u003c/sup\u003e. Anti-IL-4Rα mAb (dupilumab) has shown positive short-term effects in several case reports as well as in a small-scale real-word study\u003csup\u003e\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e, \u003cspan citationid=\"CR99\" class=\"CitationRef\"\u003e99\u003c/span\u003e, \u003cspan citationid=\"CR113\" class=\"CitationRef\"\u003e113\u003c/span\u003e, \u003cspan additionalcitationids=\"CR127 CR128 CR129 CR130\" citationid=\"CR126\" class=\"CitationRef\"\u003e126\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR131\" class=\"CitationRef\"\u003e131\u003c/span\u003e\u003c/sup\u003e. Notably, eosinophil counts and IgE levels significantly decreased in all but one patient, who transitioned from omalizumab to dupilumab with satisfactory curative effect\u003csup\u003e\u003cspan citationid=\"CR131\" class=\"CitationRef\"\u003e131\u003c/span\u003e\u003c/sup\u003e, indicating that upstream regulator of IgE, rather than IgE itself, may play a critical role in the pathogenesis of KD.\u003c/p\u003e \u003cp\u003eMepolizumab, an anti-IL-5 mAb responsible for eosinophils terminal differentiation, activation, and survival\u003csup\u003e\u003cspan citationid=\"CR132\" class=\"CitationRef\"\u003e132\u003c/span\u003e\u003c/sup\u003e, has been approved for the treatment of severe eosinophilic asthma, chronic rhinosinusitis with nasal polyps(CRSwNP), eosinophilic granulomatosis with polyangiitis (EGPA), and hypereosinophilic syndrome (HES). Recent studies have reported that mepolizumab, even at one-third of the standard dose, can effectively reduce eosinophil levels and the mass size in KD patients\u003csup\u003e\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e, \u003cspan citationid=\"CR56\" class=\"CitationRef\"\u003e56\u003c/span\u003e\u003c/sup\u003e. These effects were also observed in our patient. However, the serum levels of IgE and IL-5 did not decrease, which differs from the trends with the 300mg dose of mepolizumab\u003csup\u003e\u003cspan citationid=\"CR123\" class=\"CitationRef\"\u003e123\u003c/span\u003e\u003c/sup\u003e. Although the mechanism of KD is not fully understood, the successful application of benrelizumab (anti-IL-5Rα mAb) in a patient with asthma and atopic dermatitis further supports the important of the IL-5 signaling pathway in the pathogenesis of KD\u003csup\u003e\u003cspan citationid=\"CR133\" class=\"CitationRef\"\u003e133\u003c/span\u003e\u003c/sup\u003e. The use of these biological agents targeting type 2 inflammation suggests that the key to effectively treating KD is maintaining eosinophil homeostasis in peripheral circulation and tissues by inhibiting bone marrow to blood movement with anti-IL-5 antibodies, limiting circulation to tissue transfer with anti-IL-4 antibodies, and eliminating eosinophils in blood and tissue using anti-IL-5 receptor antibodies (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eIn conclusion, pruritus is a significant clinical feature of KD, and the time to diagnosis is often prolonged due to the variability in presentation and lack of specific diagnostic criteria. The complex nature of KD, with its overlapping features with IgG4-RD, highlights the role of Th2-mediated inflammation in its pathogenesis. Anti-IL-5, anti-IL-4, and anti-IL-5 receptor antibodies offer potential avenues for more targeted treatment. Future studies should focus on the mechanisms underlying KD and the role of Th2-driven inflammation, as well as the development of standardized treatment guidelines. Increased awareness and early diagnosis are critical in improving patient outcomes.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"350\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 24.2857%;\"\u003e\n \u003cp\u003eKD\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 75.7143%;\"\u003e\n \u003cp\u003eKimura disease\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 24.2857%;\"\u003e\n \u003cp\u003eTh2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 75.7143%;\"\u003e\n \u003cp\u003etype 2 helper T cell\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 24.2857%;\"\u003e\n \u003cp\u003ePBMC\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 75.7143%;\"\u003e\n \u003cp\u003eperipheral blood mononuclear cells\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 24.2857%;\"\u003e\n \u003cp\u003eCT\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 75.7143%;\"\u003e\n \u003cp\u003ecomputed tomography\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 24.2857%;\"\u003e\n \u003cp\u003eMRI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 75.7143%;\"\u003e\n \u003cp\u003emagnetic resonance imaging\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 24.2857%;\"\u003e\n \u003cp\u003eIgG4-RD\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 75.7143%;\"\u003e\n \u003cp\u003eIgG4-related disease\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 24.2857%;\"\u003e\n \u003cp\u003eMAb\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 75.7143%;\"\u003e\n \u003cp\u003emonoclonal antibody\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll anonymized data in this study are available\u0026nbsp;from the corresponding author upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe are grateful to the patients for their supports and participation\u0026nbsp;in this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis work was supported by the National Natural Science Foundation of China (82202054, 82270028)\u0026nbsp;and Special Fund\u0026nbsp;for Clinical Research, Zhongshan Hospital Affiliated to Fudan University (ZSLCYJ202330).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor information\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors and affiliations\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eDepartment of Allergy, Zhongshan Hospital, Fudan University, Shanghai, 200032, China.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eWenjiao Zhu, Ye Xiang, Jing Shi, Yiran Liang, Ling Ye \u0026amp; Meiling Jin\u003c/p\u003e\n\u003cp\u003eDepartment of Pathology, Zhongshan Hospital, Fudan University Shanghai, 200032, China.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eLei Zhang\u003c/p\u003e\n\u003cp\u003eDepartment of Pathology, Shanghai Ninth People\u0026rsquo;s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200011, China.\u003c/p\u003e\n\u003cp\u003eJing Zhang\u003c/p\u003e\n\u003cp\u003eDepartment of Neurosurgery, Shanghai Ninth People\u0026apos;s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200011, China.\u003c/p\u003e\n\u003cp\u003eWei Wang\u003c/p\u003e\n\u003cp\u003eHuashan Rare Disease Center and Department of Neurology, Huashan Hospital, Shanghai Medical College, National Center for Neurological Disorders, Fudan University, Shanghai 200040, China.\u003c/p\u003e\n\u003cp\u003eDingxian He\u003c/p\u003e\n\u003cp\u003eDepartment of Endocrinology and Metabolism, Peking University People\u0026apos;s Hospital, Beijing, 100044, China.\u003c/p\u003e\n\u003cp\u003eYuanping Shi\u003c/p\u003e\n\u003cp\u003eDepartment of Otolaryngology, Zhongshan Hospital, Fudan University, Shanghai, 200032, China.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eXianhui Ning\u003c/p\u003e\n\u003cp\u003eDepartment of Pulmonary and Critical Care Medicine, Shanghai Respiratory Research Institute, Zhongshan Hospital, Research Center of Allergy and Diseases, Fudan University, Shanghai, 200032, China.\u003c/p\u003e\n\u003cp\u003eLing Ye \u0026amp; Meiling Jin\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eContributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eZW, ZL, and ZJ designed the study and wrote the manuscript. WW, XY, HD, SJ, LY and SY contributed to data collection. ZW, NX, YL, JM performed the statistical analysis and interpretation of the results. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCorresponding author\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eCorrespondence to Meiling Jin, Ling Ye and Xianhui Ning\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was approved by the ethics committee of Zhongshan hospital, Fudan University\u0026nbsp;(B2019-020R)\u0026nbsp;and Shanghai Ninth People\u0026apos;s Hospital, Shanghai Jiao Tong University School of Medicine (SH9H-2024-T174-2).\u0026nbsp;Informed consent was obtained from all patients or their guardian.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent for publication of clinical details was obtained from all patients or their guardian.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll authors declare no competing interests.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eKim H-T Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz\u0026apos;s disease. 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Clin Exp Dermatol, 2024; 49(5)\u003cstrong\u003e:\u003c/strong\u003e 502-506.\u003c/li\u003e\n\u003cli\u003eYang B, Yu H, Jia M, Yao W, Diao R, Li B, et al. Successful treatment of dupilumab in Kimura disease independent of IgE: A case report with literature review. Front Immunol, 2022; 13\u003cstrong\u003e:\u003c/strong\u003e 1084879.\u003c/li\u003e\n\u003cli\u003ePelaia C, Paoletti G, Puggioni F, Racca F, Pelaia G, Canonica GW, et al. Interleukin-5 in the Pathophysiology of Severe Asthma. Front Physiol, 2019; 10\u003cstrong\u003e:\u003c/strong\u003e 1514.\u003c/li\u003e\n\u003cli\u003eSzeto VG, Chin-Yee B, Dehghani M, Rizkalla K, Licskai C, Hsia CC Successful treatment of Kimura disease with benralizumab. Ann Hematol, 2022; 101(9)\u003cstrong\u003e:\u003c/strong\u003e 2099-2100.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"orphanet-journal-of-rare-diseases","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"ojrd","sideBox":"Learn more about [Orphanet Journal of Rare Diseases](http://ojrd.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/ojrd/default.aspx","title":"Orphanet Journal of Rare Diseases","twitterHandle":"@bmc","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Kimura disease, eosinophilia, pruritus, mepolizumab, biologics","lastPublishedDoi":"10.21203/rs.3.rs-5262032/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-5262032/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eKimura disease (KD) is a rare, chronic, inflammatory disorder characterized by painless subcutaneous masses, lymphadenopathy, elevated eosinophil counts and increased serum immunoglobulin E (IgE) levels. Due to the heterogeneity and rarity, current diagnostic and treatment approaches for KD are often delayed and have limited efficacy.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMethods\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe clinicopathological data of 51 KD patients between January 2014 and December 2023 were retrieved and stratified based on age and mass size, respectively. A case ofrecurrent KD who responded well to a low dose of mepolizumab was described.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe found that pruritus occurred in 45.8% of patients, with higher rates in older patients (\u003cem\u003eP=\u003c/em\u003e0.033). Edema was observed in 29.2%, with higher prevalence in younger patients (\u003cem\u003eP=\u003c/em\u003e0.010). The mean maximum mass size was 3.1cm, varying by age group (\u003cem\u003eP=\u003c/em\u003e0.005). The mean time to diagnosis was 36 months, increasing with mass size (\u003cem\u003eP=\u003c/em\u003e0.024). Meanwhile, we reported that one of these 51 patients was successfully treated with mepolizumab at a dose of 100mg every 4 weeks.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe conclude that pruritus and edema are the two main clinical manifestation of KD, and the size of mass is related to the time of diagnosis. Biologic agents targeting the Th2 pathway show promise for KD treatment.\u003c/p\u003e","manuscriptTitle":"Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-10-29 13:06:31","doi":"10.21203/rs.3.rs-5262032/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"reviewerAgreed","content":"","date":"2025-01-18T05:41:59+00:00","index":0,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-10-25T17:54:15+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-10-22T13:39:42+00:00","index":"","fulltext":""},{"type":"submitted","content":"Orphanet Journal of Rare Diseases","date":"2024-10-19T06:07:25+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"orphanet-journal-of-rare-diseases","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"ojrd","sideBox":"Learn more about [Orphanet Journal of Rare Diseases](http://ojrd.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/ojrd/default.aspx","title":"Orphanet Journal of Rare Diseases","twitterHandle":"@bmc","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"0e29d879-89fd-4c01-a15d-b8b00d04afcd","owner":[],"postedDate":"October 29th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"under-review","subjectAreas":[],"tags":[],"updatedAt":"2024-10-29T13:06:31+00:00","versionOfRecord":[],"versionCreatedAt":"2024-10-29 13:06:31","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-5262032","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-5262032","identity":"rs-5262032","version":["v1"]},"buildId":"WrCJVZZCHTDjtuVLN7oU0","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}