Late progression of pediatric Kikuchi-Fujimoto disease to systemic lupus erythematosus : a case report

Late progression of pediatric Kikuchi-Fujimoto disease to systemic lupus erythematosus : a case report | 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 Case Report Late progression of pediatric Kikuchi-Fujimoto disease to systemic lupus erythematosus : a case report Sameh Mezri, Salma Bessioud, Karima Tlili, Hager Barakizou This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7256189/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background: Kikuchi-Fujimoto disease (KFD) is a rare benign condition affecting children. The main differential diagnoses are lymphoma and tuberculous nodes. KFD can spontaneously resolve. However, an association with systemic lupus erythematosus (SLE) has been reported, though it remains rare. Case report: We present the case of a patient with no history of immunodefiscinece, first seen at six years old for chronic cervical lymphadenopathy. Lymph node biopsy confirmed the diagnosis of KFD. Favorable evolution was obtained under corticotherapy treatment. Twelve years later, the diagnosis of SLE was made based on the appearance of skin symptoms and biological data. Throught this case, we insist to the possibility of a late transformation of KFD to a SLE and the necessity of prolonged follow-up. Conclusion: This report underscores the necessity for long-term monitoring of children diagnosed with KFD. Kikuchi disease lupus erythematosus cervical lymph node differential diagnosis case report Figures Figure 1 Figure 2 Background Kikuchi-Fujimoto disease (KFD), otherwise called necrotizing histiocytic lymphadenitis, is a generally harmless albeit at times life-altering condition primarily affecting asian young women and rarely seen in children. It usually presents with cervical lymph nodes accompanied by vague symptoms like arthralgia, fever, and/ou fatigue. Laboratory test are non specific making it a key alternative diagnosis to cancer, notably lymphoma and other causes of chronic lymphadenopathy especially for pediatric patients ( 2 ) Since its first description in 1972 ( 1 ), many cases have come to light showcasing a connection to or progression into autoinflammatory diseases, particularly systemic lupus erythematosus. The pathophysiological link between these pathologies remains unclear and subject of debate. The principales suggestions are an underlying autoimmune predisposition ( 3 , 4 ) or a genetic susceptibility by an aberrant immune response to infectious agents. In children, KFD is even more uncommon, often presenting with misleading features and a benign yet perplexing course. Several pediatric cases have been described exhibiting severe manifestations, including macrophage activation syndrome, suggesting significant immune dysregulation ( 5 , 6 ). Histological examination of lymph nodes, the only definitive diagnostic tool, reveals architectural disruption with karyorrhectic necrosis, infiltration by CD68 + histiocytes, and absence of neutrophils, distinguishing KFD from lymphoma or tuberculosis. Recent literature increasingly highlights the potential association between KFD and systemic lupus erythematosus (SLE). Approximately 13% of patients reportedly develop secondary SLE ( 4 ). This association is supported by contemporary observations, including cases where lupus follows shortly after KFD ( 7 ). Furthermore, some authors have reported overlap syndromes involving lupus and Sjögren’s syndrome succeeding KFD, reinforcing the concept of a shared immunological background ( 10 ). This case report highlights the possible association between KFD and SLE, even after a long period of time, and emphasises the importance of prolonged follow-up. The work has been reported in line with the SCARE criteria. Case Presentation A six-year-old girl with no pathological past was referred for cervical lymphadenopathy persisting for several weeks, associated with low-grade intermittent fever, fatigue and weight loss. Clinical examination revealed a firm, mobile, painless lymph node. No local inflammatory signs were found. Physical examination revealed firm, tender and mobile lymph nodes in the posterior cervical region ranging in size from 2 to 4 cm, with no changes to the overlying skin neither signs of ENT or systemic infection. Cervical ultrasonography revealed oval-shaped, hypoechoic lymph nodes with heterogeneous internal echotexture and an absent or poorly visualised hilum. Colour Doppler imaging revealed reduced or peripheral vascularisation, with no evidence of abscess or suppuration. An initial suspicion of hematologic malignancy was suspected. Laboratory investigations revealed a white blood cell count of 3200/mm³, characterised by neutropenia (neutrophils: 1,000/mm³) and relative lymphocytosis (lymphocytes : 2100/mm³). Haemoglobin and platelet levels were within normal limits. C-reactive protein levels negativ (< 5 mg/L) and the erythrocyte sedimentation rate was mildly increased to 28 mm/h. Liver transaminase levels were slightly elevated. Serological tests for the cytomegalovirus, Epstein-Barr virus, toxoplasmosis, and research of tuberculosis were negative. Chest radiography showed no abnormalities. Lymph node biopsy demonstrated disrupted architecture with abundant areas of karyorrhectic necrosis, infiltration by CD68 + histiocytes, presence of plasmacytoid monocytes, numerous apoptotic cells and an immunoblastic reaction, and absence of neutrophils or eosinophils, consistent with Kikuchi disease. (Figs. 1 , 2 ). Resolution occurred over feew weeks under corticotherapy (1mg/kg per day) Regular clinical follow-up was maintained for several years, during which two recurrences responded well to treatment with corticosteroids. At 18 years old, the patient presented again with fatigue, arthralgia, and malar rash of several months duration without improvement. Laboratory tests showed an elevated ESR (64 mm/h) with low increased CRP. We noted a normocytic anemia with moderate leucopenia and thrombocytopenia. We also noted positive proteinuria and microscopic hematuria. The immunological workup revealed a high titer of speckled antinuclear antibodies (ANA) (1:640); positive anti-double-stranded DNA (dsDNA) antibodies; and low complement levels (C3: 0.7 g/L; C4: 0.06 g/L). The direct Coombs test was positive (Table). Table Biological findings Parameter Result Reference Range Comment Erythrocyte Sedimentation Rate (ESR) 64 mm/h < 20 mm/h Elevated C-Reactive Protein (CRP) 09 mg/L < 5 mg/L Mildly increased Hemoglobin 9.6 g/dL 12–16 g/dL (female) Anemia White Blood Cell Count 3100 /mm³ 4,000–10,000 /mm³ Leukopenia Platelet Count 105x10 3 /mm³ 150,000–400,000/mm³ Thrombocytopenia Proteinuria (24h) 1.2 g/24 h < 150 mg/24 h Significant Hematuria (microscopic) Present Absent Abnormal Antinuclear Antibodies (ANA) 1:640(speckled) < 1:80 Strongly positive Anti-dsDNA Antibodies Positive Negative Complement C3 0.7 g/L 0.9–1.8 g/L Decreased Complement C4 0.06 g/L 0.1–0.4 g/L Decreased Direct Coombs Test Positive Negative According to the EULAR/ACR criteria, the diagnosis of systemic lupus erythematosus was establiashed. Liver function tests, cardiac exploration (echocardiogram), thoracic exploration (chest X-ray) and electrolyte levels, were normal and no evidence of significant visceral involvement was noted at this stage. Patient was referred to internal medicine department. The treatment associated 200 mg daily of hydroxychloroquine and 0.5 mg per kg per day of oral corticosteroids (prednisone*) due to hematologic and immunologic activity. Because of non signifant visceral involvment, no immunosuppressive therapy was prescribed. Regular clinical and laboratory monitoring was prescribed. Discussion Kikuchi-Fujimoto disease (KFD) is a rare form of necrotizing histiocytic lymphadenitis. It’s etiologie is remain unclear. Predominantly affecting young Asian femal, many pediatric cases are recently more reported, expanding the demographic spectrum of this entity ( 11 , 12 ). In typical cases, KFD manifests as firm, tender, unilateral cervical lymphadenopathy, accompanied by low-grade fever or fever associated with asthenia, loss of appetite, and arthralgia. Histological examination confirms the diagnosis by revealing an alteration in lymph node architecture with the presence of karyorrheic necrosis without neutrophils and a proliferation of CD68 + histiocytes ( 13 ). In children, KFD remains exceptional and poses a significant diagnostic challenge. A pediatric series by Wang and al. ( 12 ) highlighted the misleading clinical presentation and the frequent use of invasive procedures to exclude lymphoma. More recently, Liu C and al ( 5 ) and Shen Zc and al ( 6 ) described pediatric forms complicated by macrophage activation syndrome, suggesting an exaggerated immune response possibly related to autoimmune dysregulation. The possible association between KFD and systemic lupus erythematosus (SLE) has been discussed in recent studies and several authors suggest that KFD may be an early stage/manifestation of SLE. However pathophysiological mechanisms remain unclear ( 8 ). The temporal relation between the two conditions varies ; KFD may precede, coincide with, or follow the diagnosis of SLE. Tarabishi S and al ( 3 ) estimated that approximately 13% of KFD cases eventually develop SLE. More recent reports support this observation. Yousefi and al ( 7 ) described a case of near-simultaneous onset in an adolescent, while Harrison and al ( 8 ) reported an initial KFD presentation in a young girl who developed SLE shortly thereafter. In our case, the 12-year interval between the initial diagnosis of KFD and the onset of SLE emphasizes the potentially latent nature of this progression. Such delayed evolution is rarely documented. Deb and al ( 14 ), in a retrospective case series, noted that SLE onset could be delayed by several years, and stressed the importance of long-term follow-up even in cases with complete clinical remission. Similarly, Owczarczyk-Saczonek and al ( 15 ) reported the emergence of chronic cutaneous lupus following isolated KFD, suggesting a possible underlying immunological continuum. These observations support the hypothesis of a shared immunopathogenic mechanism. KFD may reflect a transient phase of immune activation in genetically predisposed individuals. Risk factors may include complement abnormalities, transient antinuclear autoantibodies, or a personal/family history of autoimmunity. Some authors have proposed that the pathogenesis of KFD may involve activation and proliferation of CD8 + T lymphocytes in response to autoimmune, infectious, or other unidentified triggers ( 5 ). Several studies have suggested that cytokines like interferon-gamma and interleukin-6 may play a role in the pathogenesis of Kikuchi-Fujimoto disease. Still, the underlying immune processes haven’t been fully worked out yet ( 16 ). There are no standardized recommendations for KFD follow-up in paedratric people. However, many studies underline the value of long-term clinical and immunological monitoring particularly in female patients due to the frequency of emergence of autoimmune conditions like lupus. Clinicians should stay vigilant for unexplained signs such as fatigue, arthralgia, or skin manifestations, and recent assess of ANA levels, complement activity or a perturbation of hematological parameters. In general, KFD resolves on its own and doesn’t usually require more than supportive care. Things become more complex when a patient later develops systemic lupus erythematosus. In our case, hydroxychloroquine combined with corticosteroids led to noticeable improvement, echoing what has been reported in other early lupus cases that followed KFD ( 7 , 8 ). This case serves as a reminder of how rare KFD is in the pediatric setting and why these patients shouldn't be lost to long follow-up. It also highlight the hypothesis of a link between KFD and lupus and shows why a past history of KFD matters in assessing future autoimmune risk. Conclusion Although Kikuchi disease is a condition that should be considered among the diagnoses to be considered in cases of chronic adenopathy in children. Although it is known to resolve spontaneously, its currently recognized relationship with SLE warrants long-term follow-up to detect any early transformation even after a long period of inactivity This case underscores the relevance of long-term monitoring and contributes valuable insight into the possible immunological continuum linking KFD to autoimmune disorders. Abbreviations ANA antinuclear antibodies EULAR/ACR The American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) ESR erythrocytes sedimentation rate SLE systemic lupus erythematosus KFD Kikuchi-Fujimoto disease mg/l milligram per liter Kg kilogram mm/l milliliter per liter ENT ear throat and nose CRP C-reactive protein Declarations Ethical Approval : Ethical approval is not applicable/waived at our institution. Due to the specific nature of case reports, which involve detailed descriptions of observations and interventions that have already been conducted on patients, as opposed to prospective studies involving planned interventions, our institution does not require formal ethical approval for such cases. We recognize the importance of ethics in medical research and are fully committed to upholding ethical standards in our medical and research practices. Consent for pulication Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for the Editor/reviewers to review upon request. Competings interest The authors declare that they have no competing interests Finding No fundings Author Contribution S.B and H.B : wrote the main manuscript textK.T: prepared figures (1 and 2)S.M: supervised and validate the projectAll authors reviewed and approved the manuscript Acknowledgement We thank the patient and his family for allowing us to share his information Availability of data and materials : Not applicable References Dorfman RF, Berry GJ (1988) Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 5(4):329–45. Norris AH, Krasinskas AM, Salhany KE, Gluckman SJ (1996) Kikuchi-Fujimoto disease: a benign cause of fever and lymphadenopathy. Am J Med 101(4):401–5. doi: 10.1016/S0002-9343(96)00231-8 . Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2024) Kikuchi-Fujimoto Disease: analysis of 244 cases. Clin Rheumatol 26(1):50–4. doi: 10.1007/s10067-006-0230-5 . Tarabichi S, Nicola AA, Radu L, Enache S, Busioc CL, Moldovianu A (2022) Kikuchi-Fujimoto disease and systemic lupus erythematosus – an enigmatic association for clinicians and pathologists: Case report and short literature review. Romanian Journal of Rhinology 12(45):11–21. doi: 10.2478/rjr-2022-0003 Liu C, Jin Y, Huang H, Ding F, Yang Z, Xu X, Bao S, Ma J, Jin Y (2022) Kikuchi-Fujimoto disease as the initial manifestation of systemic lupus erythematosus complicated with macrophage activation syndrome: two case reports and a review of literature. BMC Pediatr 22(1):673. doi: 10.1186/s12887-022-03703-6 . Shen Z, Ling J, Zhu X, Yang J, He T (2023) Macrophage activation syndrome in children with Kikuchi-Fujimoto disease. Pediatr Rheumatol Online J 21(1):10. doi: 10.1186/s12969-023-00788-w . Yousefi M, Rukerd MRZ, Binafar H, Shoaie S, Mirkamali H, Pourzand P (2023) The co-occurrence of Kikuchi-Fujimoto disease and systemic lupus erythematosus: a case report. J Med Case Rep 17(1):448. doi: 10.1186/s13256-023-04186-4 . Harrison J, Sukumaran S, Vijayan V (2023) Systemic Lupus Erythematosus Lymphadenopathy Presenting as Kikuchi-Fujimoto Disease in an Adolescent. Cureus 15(2):e35304. doi: 10.7759/cureus.35304 . Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R (2019) European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol 71(9):1400–1412. doi: 10.1002/art.40930 . Cindy C, Wibowo SAK, Ariane A, Hidayat R (2024) Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren's Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report. Acta Med Indones 56(2):210–217. PMID: 39010763 LiLin YC, Huang HH, Nong BR, Liu PY, Chen YY, Huang YF (2019) Pediatric Kikuchi-Fujimoto disease: A clinicopathologic study and the therapeutic effects of hydroxychloroquine. J Microbiol Immunol Infect 52(3):395–401. doi: 10.1016/j.jmii.2017.08.023 . Wang TJ, Yang YH, Lin YT, Chiang BL (2004) Kikuchi-Fujimoto disease in children: clinical features and disease course. J Microbiol Immunol Infect 37(4):219–224. PMID: 15340649. Perry AM, Choi SM (2018) Kikuchi-Fujimoto Disease: A Review. Arch Pathol Lab Med 142(11):1341–1346. Deb A, Fernandez V, Kilinc E, Bahmad HF, Camps NS, Sriganeshan V (2024) Kikuchi-Fujimoto Disease: A Case Series and Review of the Literature. Diseases 12(11):271. doi: 10.3390/diseases12110271 . Owczarczyk-Saczonek A, Jóźwicka A. Chronic Cutaneous (2024) Lupus Erythematosus in a Patient with History of KFD. EMJ Dermatol 12(1):61–63. https://doi.org/10.33590/emjdermatol/FWWC4329 Zuo Y, Foshat M, Qian YW, Kelly B, Harper B, Karnath B (2012) A Rare Case of Kikuchi Fujimoto's Disease with Subsequent Development of Systemic Lupus Erythematosus. Case Rep Rheumatol:325062. doi: 10.1155/2012/325062 . Epub 2012 Dec 30. Additional Declarations No competing interests reported. Supplementary Files scarechecklist.docx Cite Share Download PDF Status: Posted Version 1 posted 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. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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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-7256189","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":495836546,"identity":"6c8bbe28-aa88-4bf8-a857-6d40028c3810","order_by":0,"name":"Sameh Mezri","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA2UlEQVRIiWNgGAWjYJACZgjJfICBsQHEOEC0FrYEiBYgTaQWBh4D4rTIt59OfFyYY2Nv3s7zTeLnDhs5BjbeB3i1GJzJ3Ww8c1sas8xh3m2SvWfSjBnY2A3wa2HI3SbNu+0wmwQz7zYJ3rbDiQ3ybQQc1v92+2/ebf95JJh5nkn+BWlhY8OvheFG7jag+QckgFrYpHmJ0WJw4+1moMOSDSSY2YytZdvSjNkIaZHvz934mXebnb0E/+GHN9+22cjxE3QYEmCRAJEkaABG6QdSVI+CUTAKRsHIAQCZpztVep8DkQAAAABJRU5ErkJggg==","orcid":"","institution":"Military Hospital of Tunis","correspondingAuthor":true,"prefix":"","firstName":"Sameh","middleName":"","lastName":"Mezri","suffix":""},{"id":495836547,"identity":"19dd2541-5b9b-469b-835e-6e26b6e4bcc6","order_by":1,"name":"Salma Bessioud","email":"","orcid":"","institution":"Military Hospital of Tunis","correspondingAuthor":false,"prefix":"","firstName":"Salma","middleName":"","lastName":"Bessioud","suffix":""},{"id":495836548,"identity":"3c6cfe52-ece4-459f-a3d0-50edb556fda3","order_by":2,"name":"Karima Tlili","email":"","orcid":"","institution":"Military Hospital of Tunis","correspondingAuthor":false,"prefix":"","firstName":"Karima","middleName":"","lastName":"Tlili","suffix":""},{"id":495836549,"identity":"fb7673aa-090e-43fd-a9ba-c48659ab6043","order_by":3,"name":"Hager Barakizou","email":"","orcid":"","institution":"Military Hospital of Tunis","correspondingAuthor":false,"prefix":"","firstName":"Hager","middleName":"","lastName":"Barakizou","suffix":""}],"badges":[],"createdAt":"2025-07-30 20:23:17","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7256189/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7256189/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":88503183,"identity":"aa60795d-8c06-4570-a745-37ee186fbd4c","added_by":"auto","created_at":"2025-08-07 07:02:52","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":1005901,"visible":true,"origin":"","legend":"\u003cp\u003e(HEX4) The lymph node is enlarged with architectural distorsion including large serpiginous shaped areas of pale staining (A), (HEX20) the pale areas are comprised of mononuclear cells amorphous necrotic debris and abundant karyorrhectic debris (B).\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-7256189/v1/4f5ccf427cc5708de02882c6.png"},{"id":88503182,"identity":"66c04bea-f2c2-436a-a522-8d1bd92f3642","added_by":"auto","created_at":"2025-08-07 07:02:52","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":39208,"visible":true,"origin":"","legend":"\u003cp\u003eCD 68 highlights abundant histiocytes surrounding areas of necrosis\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7256189/v1/5141d17a59e056fe5975e01c.jpg"},{"id":93324634,"identity":"6caecd08-5567-4b0e-81cf-4481dbc5b628","added_by":"auto","created_at":"2025-10-12 08:16:23","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":5466878,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7256189/v1/e33762e4-7f31-4f20-92a1-33cf4b114c19.pdf"},{"id":88499903,"identity":"939056ac-d50f-41d0-8ed8-d1519b4b0adc","added_by":"auto","created_at":"2025-08-07 06:46:52","extension":"docx","order_by":0,"title":"","display":"","copyAsset":false,"role":"supplement","size":29387,"visible":true,"origin":"","legend":"","description":"","filename":"scarechecklist.docx","url":"https://assets-eu.researchsquare.com/files/rs-7256189/v1/4e667055d0639f9be946da3d.docx"}],"financialInterests":"No competing interests reported.","formattedTitle":"Late progression of pediatric Kikuchi-Fujimoto disease to systemic lupus erythematosus : a case report","fulltext":[{"header":"Background","content":"\u003cp\u003eKikuchi-Fujimoto disease (KFD), otherwise called necrotizing histiocytic lymphadenitis, is a generally harmless albeit at times life-altering condition primarily affecting asian young women and rarely seen in children. It usually presents with cervical lymph nodes accompanied by vague symptoms like arthralgia, fever, and/ou fatigue. Laboratory test are non specific making it a key alternative diagnosis to cancer, notably lymphoma and other causes of chronic lymphadenopathy especially for pediatric patients (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e)\u003c/p\u003e\u003cp\u003eSince its first description in 1972 (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e), many cases have come to light showcasing a connection to or progression into autoinflammatory diseases, particularly systemic lupus erythematosus. The pathophysiological link between these pathologies remains unclear and subject of debate. The principales suggestions are an underlying autoimmune predisposition (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e) or a genetic susceptibility by an aberrant immune response to infectious agents.\u003c/p\u003e\u003cp\u003eIn children, KFD is even more uncommon, often presenting with misleading features and a benign yet perplexing course. Several pediatric cases have been described exhibiting severe manifestations, including macrophage activation syndrome, suggesting significant immune dysregulation (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). Histological examination of lymph nodes, the only definitive diagnostic tool, reveals architectural disruption with karyorrhectic necrosis, infiltration by CD68 + histiocytes, and absence of neutrophils, distinguishing KFD from lymphoma or tuberculosis.\u003c/p\u003e\u003cp\u003eRecent literature increasingly highlights the potential association between KFD and systemic lupus erythematosus (SLE). Approximately 13% of patients reportedly develop secondary SLE (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). This association is supported by contemporary observations, including cases where lupus follows shortly after KFD (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e). Furthermore, some authors have reported overlap syndromes involving lupus and Sjögren’s syndrome succeeding KFD, reinforcing the concept of a shared immunological background (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eThis case report highlights the possible association between KFD and SLE, even after a long period of time, and emphasises the importance of prolonged follow-up. The work has been reported in line with the SCARE criteria.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA six-year-old girl with no pathological past was referred for cervical lymphadenopathy persisting for several weeks, associated with low-grade intermittent fever, fatigue and weight loss. Clinical examination revealed a firm, mobile, painless lymph node. No local inflammatory signs were found.\u003c/p\u003e\u003cp\u003ePhysical examination revealed firm, tender and mobile lymph nodes in the posterior cervical region ranging in size from 2 to 4 cm, with no changes to the overlying skin neither signs of ENT or systemic infection. Cervical ultrasonography revealed oval-shaped, hypoechoic lymph nodes with heterogeneous internal echotexture and an absent or poorly visualised hilum. Colour Doppler imaging revealed reduced or peripheral vascularisation, with no evidence of abscess or suppuration.\u003c/p\u003e\u003cp\u003eAn initial suspicion of hematologic malignancy was suspected. Laboratory investigations revealed a white blood cell count of 3200/mm³, characterised by neutropenia (neutrophils: 1,000/mm³) and relative lymphocytosis (lymphocytes : 2100/mm³). Haemoglobin and platelet levels were within normal limits. C-reactive protein levels negativ (\u0026lt; 5 mg/L) and the erythrocyte sedimentation rate was mildly increased to 28 mm/h. Liver transaminase levels were slightly elevated.\u003c/p\u003e\u003cp\u003eSerological tests for the cytomegalovirus, Epstein-Barr virus, toxoplasmosis, and research of tuberculosis were negative. Chest radiography showed no abnormalities.\u003c/p\u003e\u003cp\u003eLymph node biopsy demonstrated disrupted architecture with abundant areas of karyorrhectic necrosis, infiltration by CD68 + histiocytes, presence of plasmacytoid monocytes, numerous apoptotic cells and an immunoblastic reaction, and absence of neutrophils or eosinophils, consistent with Kikuchi disease. (Figs.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e, \u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eResolution occurred over feew weeks under corticotherapy (1mg/kg per day)\u003c/p\u003e\u003cp\u003eRegular clinical follow-up was maintained for several years, during which two recurrences responded well to treatment with corticosteroids. At 18 years old, the patient presented again with fatigue, arthralgia, and malar rash of several months duration without improvement. Laboratory tests showed an elevated ESR (64 mm/h) with low increased CRP. We noted a normocytic anemia with moderate leucopenia and thrombocytopenia.\u003c/p\u003e\u003cp\u003eWe also noted positive proteinuria and microscopic hematuria. The immunological workup revealed a high titer of speckled antinuclear antibodies (ANA) (1:640); positive anti-double-stranded DNA (dsDNA) antibodies; and low complement levels (C3: 0.7 g/L; C4: 0.06 g/L). The direct Coombs test was positive (Table).\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eTable Biological findings\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cdiv class=\"gridtable\"\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\u003ctable float=\"No\" id=\"Taba\" border=\"1\"\u003e\u003ccolgroup cols=\"4\"\u003e\u003c/colgroup\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u003cp\u003eParameter\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eResult\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eReference Range\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eComment\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eErythrocyte Sedimentation Rate (ESR)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e64 mm/h\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026lt; 20 mm/h\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eElevated\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eC-Reactive Protein (CRP)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e09 mg/L\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026lt; 5 mg/L\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eMildly increased\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eHemoglobin\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e9.6 g/dL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e12–16 g/dL (female)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eAnemia\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eWhite Blood Cell Count\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3100 /mm³\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e4,000–10,000 /mm³\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eLeukopenia\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePlatelet Count\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e105x10\u003csup\u003e3\u003c/sup\u003e/mm³\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e150,000–400,000/mm³\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eThrombocytopenia\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eProteinuria (24h)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e1.2 g/24 h\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026lt; 150 mg/24 h\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eSignificant\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eHematuria (microscopic)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003ePresent\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eAbsent\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eAbnormal\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAntinuclear Antibodies (ANA)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e1:640(speckled)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026lt; 1:80\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eStrongly positive\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAnti-dsDNA Antibodies\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003ePositive\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eNegative\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eComplement C3\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e0.7 g/L\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e0.9–1.8 g/L\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eDecreased\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eComplement C4\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e0.06 g/L\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e0.1–0.4 g/L\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eDecreased\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eDirect Coombs Test\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003ePositive\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eNegative\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/table\u003e\u003c/div\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eAccording to the EULAR/ACR criteria, the diagnosis of systemic lupus erythematosus was establiashed. Liver function tests, cardiac exploration (echocardiogram), thoracic exploration (chest X-ray) and electrolyte levels, were normal and no evidence of significant visceral involvement was noted at this stage.\u003c/p\u003e\u003cp\u003ePatient was referred to internal medicine department. The treatment associated 200 mg daily of hydroxychloroquine and 0.5 mg per kg per day of oral corticosteroids (prednisone*) due to hematologic and immunologic activity. Because of non signifant visceral involvment, no immunosuppressive therapy was prescribed. Regular clinical and laboratory monitoring was prescribed.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eKikuchi-Fujimoto disease (KFD) is a rare form of necrotizing histiocytic lymphadenitis. It\u0026rsquo;s etiologie is remain unclear. Predominantly affecting young Asian femal, many pediatric cases are recently more reported, expanding the demographic spectrum of this entity (\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eIn typical cases, KFD manifests as firm, tender, unilateral cervical lymphadenopathy, accompanied by low-grade fever or fever associated with asthenia, loss of appetite, and arthralgia. Histological examination confirms the diagnosis by revealing an alteration in lymph node architecture with the presence of karyorrheic necrosis without neutrophils and a proliferation of CD68\u0026thinsp;+\u0026thinsp;histiocytes (\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eIn children, KFD remains exceptional and poses a significant diagnostic challenge. A pediatric series by Wang and al. (\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e) highlighted the misleading clinical presentation and the frequent use of invasive procedures to exclude lymphoma. More recently, Liu C and al (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e) and Shen Zc and al (\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e) described pediatric forms complicated by macrophage activation syndrome, suggesting an exaggerated immune response possibly related to autoimmune dysregulation.\u003c/p\u003e\u003cp\u003eThe possible association between KFD and systemic lupus erythematosus (SLE) has been discussed in recent studies and several authors suggest that KFD may be an early stage/manifestation of SLE. However pathophysiological mechanisms remain unclear (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e). The temporal relation between the two conditions varies ; KFD may precede, coincide with, or follow the diagnosis of SLE. Tarabishi S and al (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e) estimated that approximately 13% of KFD cases eventually develop SLE. More recent reports support this observation. Yousefi and al (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e) described a case of near-simultaneous onset in an adolescent, while Harrison and al (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e) reported an initial KFD presentation in a young girl who developed SLE shortly thereafter.\u003c/p\u003e\u003cp\u003eIn our case, the 12-year interval between the initial diagnosis of KFD and the onset of SLE emphasizes the potentially latent nature of this progression. Such delayed evolution is rarely documented. Deb and al (\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e), in a retrospective case series, noted that SLE onset could be delayed by several years, and stressed the importance of long-term follow-up even in cases with complete clinical remission. Similarly, Owczarczyk-Saczonek and al (\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e) reported the emergence of chronic cutaneous lupus following isolated KFD, suggesting a possible underlying immunological continuum.\u003c/p\u003e\u003cp\u003eThese observations support the hypothesis of a shared immunopathogenic mechanism. KFD may reflect a transient phase of immune activation in genetically predisposed individuals. Risk factors may include complement abnormalities, transient antinuclear autoantibodies, or a personal/family history of autoimmunity.\u003c/p\u003e\u003cp\u003eSome authors have proposed that the pathogenesis of KFD may involve activation and proliferation of CD8\u0026thinsp;+\u0026thinsp;T lymphocytes in response to autoimmune, infectious, or other unidentified triggers (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eSeveral studies have suggested that cytokines like interferon-gamma and interleukin-6 may play a role in the pathogenesis of Kikuchi-Fujimoto disease. Still, the underlying immune processes haven\u0026rsquo;t been fully worked out yet (\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eThere are no standardized recommendations for KFD follow-up in paedratric people. However, many studies underline the value of long-term clinical and immunological monitoring particularly in female patients due to the frequency of emergence of autoimmune conditions like lupus. Clinicians should stay vigilant for unexplained signs such as fatigue, arthralgia, or skin manifestations, and recent assess of ANA levels, complement activity or a perturbation of hematological parameters.\u003c/p\u003e\u003cp\u003eIn general, KFD resolves on its own and doesn\u0026rsquo;t usually require more than supportive care. Things become more complex when a patient later develops systemic lupus erythematosus. In our case, hydroxychloroquine combined with corticosteroids led to noticeable improvement, echoing what has been reported in other early lupus cases that followed KFD (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eThis case serves as a reminder of how rare KFD is in the pediatric setting and why these patients shouldn't be lost to long follow-up. It also highlight the hypothesis of a link between KFD and lupus and shows why a past history of KFD matters in assessing future autoimmune risk.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eAlthough Kikuchi disease is a condition that should be considered among the diagnoses to be considered in cases of chronic adenopathy in children. Although it is known to resolve spontaneously, its currently recognized relationship with SLE warrants long-term follow-up to detect any early transformation even after a long period of inactivity\u003c/p\u003e\u003cp\u003eThis case underscores the relevance of long-term monitoring and contributes valuable insight into the possible immunological continuum linking KFD to autoimmune disorders.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eANA\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eantinuclear antibodies\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eEULAR/ACR\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eThe American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR)\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eESR\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eerythrocytes sedimentation rate\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eSLE\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003esystemic lupus erythematosus\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eKFD\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eKikuchi-Fujimoto disease\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003emg/l\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003emilligram per liter\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eKg\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003ekilogram\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003emm/l\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003emilliliter per liter\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eENT\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eear throat and nose\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eCRP\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eC-reactive protein\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003e\u003cb\u003eEthical Approval\u003c/b\u003e :\u003c/strong\u003e\u003cp\u003eEthical approval is not applicable/waived at our institution. Due to the specific nature of case reports, which involve detailed descriptions of observations and interventions that have already been conducted on patients, as opposed to prospective studies involving planned interventions, our institution does not require formal ethical approval for such cases. We recognize the importance of ethics in medical research and are fully committed to upholding ethical standards in our medical and research practices.\u003c/p\u003e\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eConsent for pulication\u003c/strong\u003e\u003cp\u003eWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for the Editor/reviewers to review upon request.\u003c/p\u003e\u003c/p\u003e\u003cp\u003e\u003ch2\u003eCompetings interest\u003c/h2\u003e\u003cp\u003eThe authors declare that they have no competing interests\u003c/p\u003e\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eFinding\u003c/strong\u003e\u003cp\u003eNo fundings\u003c/p\u003e\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eS.B and H.B : wrote the main manuscript textK.T: prepared figures (1 and 2)S.M: supervised and validate the projectAll authors reviewed and approved the manuscript\u003c/p\u003e\u003ch2\u003eAcknowledgement\u003c/h2\u003e\u003cp\u003eWe thank the patient and his family for allowing us to share his information\u003c/p\u003e\u003ch2\u003eAvailability of data and materials :\u003c/h2\u003e\u003cp\u003eNot applicable\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eDorfman RF, Berry GJ (1988) Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 5(4):329\u0026ndash;45.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eNorris AH, Krasinskas AM, Salhany KE, Gluckman SJ (1996) Kikuchi-Fujimoto disease: a benign cause of fever and lymphadenopathy. Am J Med 101(4):401\u0026ndash;5. doi: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/S0002-9343(96)00231-8\u003c/span\u003e\u003cspan address=\"10.1016/S0002-9343(96)00231-8\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2024) Kikuchi-Fujimoto Disease: analysis of 244 cases. Clin Rheumatol 26(1):50\u0026ndash;4. doi: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s10067-006-0230-5\u003c/span\u003e\u003cspan address=\"10.1007/s10067-006-0230-5\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eTarabichi S, Nicola AA, Radu L, Enache S, Busioc CL, Moldovianu A (2022) Kikuchi-Fujimoto disease and systemic lupus erythematosus \u0026ndash; an enigmatic association for clinicians and pathologists: Case report and short literature review. Romanian Journal of Rhinology 12(45):11\u0026ndash;21. doi: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.2478/rjr-2022-0003\u003c/span\u003e\u003cspan address=\"10.2478/rjr-2022-0003\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eLiu C, Jin Y, Huang H, Ding F, Yang Z, Xu X, Bao S, Ma J, Jin Y (2022) Kikuchi-Fujimoto disease as the initial manifestation of systemic lupus erythematosus complicated with macrophage activation syndrome: two case reports and a review of literature. 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Case Rep Rheumatol:325062. doi: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1155/2012/325062\u003c/span\u003e\u003cspan address=\"10.1155/2012/325062\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e. Epub 2012 Dec 30.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"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":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Kikuchi disease, lupus erythematosus, cervical lymph node, differential diagnosis, case report","lastPublishedDoi":"10.21203/rs.3.rs-7256189/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7256189/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground:\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eKikuchi-Fujimoto disease (KFD) is a rare benign condition affecting children. The main differential diagnoses are lymphoma and tuberculous nodes. KFD can spontaneously resolve. However, an association with systemic lupus erythematosus (SLE) has been reported, though it remains rare.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase report:\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe present the case of a patient with no history of immunodefiscinece, first seen at six years old for chronic cervical lymphadenopathy. Lymph node biopsy confirmed the diagnosis of KFD. Favorable evolution was obtained under corticotherapy treatment. Twelve years later, the diagnosis of SLE was made based on the appearance of skin symptoms and biological data. Throught this case, we insist to the possibility of a late transformation of KFD to a SLE and the necessity of prolonged follow-up.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion:\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis report underscores the necessity for long-term monitoring of children diagnosed with KFD.\u003c/p\u003e","manuscriptTitle":"Late progression of pediatric Kikuchi-Fujimoto disease to systemic lupus erythematosus : a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-08-07 06:46:47","doi":"10.21203/rs.3.rs-7256189/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"b979f0ba-09f8-46b2-960f-af4f6ccf1b5c","owner":[],"postedDate":"August 7th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-10-12T08:08:10+00:00","versionOfRecord":[],"versionCreatedAt":"2025-08-07 06:46:47","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7256189","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7256189","identity":"rs-7256189","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}