Pure Red Cell Aplasia Associated with Systemic Lupus Erythematosus in Children Response to Rituximab– A Case Report and Literature Review

Pure Red Cell Aplasia Associated with Systemic Lupus Erythematosus in Children Response to Rituximab– A Case Report and Literature Review | 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 Pure Red Cell Aplasia Associated with Systemic Lupus Erythematosus in Children Response to Rituximab– A Case Report and Literature Review Haiyan Wang, Rongqiong Ou, Bihong Zhang, Sha Li, Weiping Tan This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3628283/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 Purpose : This study analyzes the clinical presentation of SLE-associated PRCA in a pediatric patient at our institution and evaluates rituximab's therapeutic efficacy. Methods: To summarize the clinical characteristics of SLE related PRCA in a child diagnosed in our hospital by literature review, and discuss the efficacy of rituximab in treating this disease. Results : A 12-year-old girl from Guangdong, China, was admitted to our hospital with the chief complaint of "3 years of SLE history and anemia persisting for 16 days." She received her initial SLE diagnosis at 8 years and 5 months of age. Intravenous cyclophosphamide pulse and methylprednisolone (MP) pulse therapy were administered, followed by maintenance treatment with mycophenolate mofetil (MMF). During her follow-up, she maintained an SLE Disease Activity Index (SLEDAI) score of "0." At 12 years of age, the patient developed progressive anemia, A bone marrow biopsy confirmed the diagnosis of pure red cell aplastic anemia. The anemia did not respond to immunoglobulin, corticosteroids, or cyclosporine A. Subsequently, rituximab was administered, leading to a gradual normalization of her hemoglobin and reticulocyte count. She was subsequently treated with MMF for maintenance, with an SLEDAI score of "0" during the follow-up period. Conclusion : Corticosteroids have traditionally been the mainstay of treatment for SLE-related PRCA, and the combination of cyclosporine and corticosteroids has shown promise in improving remission rates and reducing recurrence rates. The administration of rituximab, followed by sequential MMF use in the presented case, demonstrated significant efficacy. rituximab systemic lupus erythematosus pure red cell aplastic anemia Figures Figure 1 Figure 2 What is known SLE-associated PRCA is a rare hematological complication that presents significant therapeutic challenges. Corticosteroids have traditionally been the mainstay of treatment for SLE-related PRCA, and the combination of cyclosporine and corticosteroids has shown promise in improving remission rates and reducing recurrence rates. What is new Should the dual regimen of glucocorticoids and cyclosporine fail to elicit the desired therapeutic response, alternative approaches, such as rituximab followed by maintenance with mycophenolate mofetil, may be employed. Introduction Systemic Lupus Erythematosus (SLE) is a multifaceted autoimmune disorder with the potential to affect multiple organ systems. Involvement of the hematological system is frequently observed, often manifesting initially with symptoms such as anemia or immune thrombocytopenia [ 40 ]. Among these hematological complications, pure red cell aplastic anemia (PRCA) emerges as a particularly rare subtype of normocytic, normochromic anemia, distinguished by a deficiency in bone marrow erythropoiesis. PRCA is categorically divided into congenital and acquired forms, with the latter encompassing primary PRCA and secondary PRCA – the latter of which recognizes SLE as an etiological factor [ 28 ]. The treatment of SLE-associated PRCA, despite its clinical significance, is predominantly documented through case reports and lacks comprehensive guidelines. This study endeavors to elucidate the management of SLE-related PRCA, presenting a case report of a patient who developed PRCA subsequent to the cessation of SLE medication, complemented by a review of pertinent literature. Materials and methods The clinical characteristics of the Patient were analyzed. Clinical data, including age, sex, initial symptoms, lab tests and therapy were collected. The study was conducted in accordance with the principles outlined in the Declaration of Helsinki with approval from the ethics committee of Sun Yat-sen Memorial Hospital, Sun Yat-Sen University [Ethical number: SYSKY-2023-1134-01]. Written informed consent was obtained from the Patient or her parents. Patient’ medical report In August 2022, a 12-year-old girl from Maoming, Guangdong, was readmitted to our institution with the primary complaint of a three-year history of Systemic Lupus Erythematosus (SLE) and a 16-day duration of anemia. She initially presented to our hospital at 8 years and 5 months old with symptoms of skin rashes and arthralgia, her diagnostic evaluations yielded a high anti-nuclear antibody (ANA) titer of 1:1280, positive anti-double-stranded DNA (anti-ds-DNA), and positive anti-ribosomal, anti-Smith, anti-ribonucleoprotein, and anti-nucleosome antibodies. Complement levels were substantially reduced (C3: 0.225 g/L, C4: 0.031 g/L), with a complete blood count revealing leukopenia (WBC: 2.98 ×10^9/L), hemoglobin of 116 g/L, and platelets at 183 × 10^12/L. A renal ultrasound exhibited effusions and synovial thickening in the left ankle and knee, without evidence of proteinuria or hematuria. The patient met the 2017 ACR criteria for SLE diagnosis and received treatment with cyclophosphamide (CTX) (10 mg/kg/day for two days, biweekly for a total of eight doses) and methylprednisolone (0.5 g/day for three days), which normalized the erythrocyte sedimentation rate (ESR) and 24-hour urinary protein levels. All immunologic markers including ANA, anti-ds-DNA, and complement levels returned to normal, alongside a negative urine microalbumin test. Maintenance therapy with mycophenolate mofetil was commenced, with the patient maintaining an SLE Disease Activity Index (SLEDAI) score of 0 at follow-up. At the age of 10 years and 5 months, the patient's medications, including mycophenolate mofetil, prednisone, and hydroxychloroquine, were discontinued and replaced with methotrexate. However, at 12 years of age, blood tests revealed anemia with hemoglobin levels ranging from 54 to 72 g/L and an elevated ESR (27 mm/h). Further serological examinations demonstrated positive results for anti-nuclear antibodies, anti-ds-DNA antibodies, anti-nuclear U1-nRNP antibodies, Smith antibodies, anti-nucleosome antibodies, and anti-nucleolar antibodies. Complement C3 was 0.65 g/L, and erythropoietin levels were elevated at 652.0 mIU/mL. A positive direct Coombs test led to the diagnosis of "SLE complicated with immune hemolytic anemia." Intravenous immunoglobulin (1 g/kg) was administered without yielding any response. Bone marrow examination revealed active bone marrow hyperplasia with reduced proliferation of the red giant cell lineage (Fig. 1 A- 1 B) and free-type homogeneous bodies accounting for 1%. Bone marrow biopsy demonstrated significant inhibition of erythroid hyperplasia (Fig. 1 C- 1 D), leading to the consideration of pure red cell aplastic anemia (PRCA). Lymphocyte subgroup analysis indicated elevated proportions of CD20 + B cells. Subsequently, the patient received methylprednisolone pulse therapy and cyclosporine, but her hemoglobin levels failed to improve, necessitating blood transfusions. Rituximab (375 mg/m^2/week × 4 doses) was then administered, leading to gradual improvements in hemoglobin levels. Subsequent management included the sequential use of mycophenolate mofetil (20–30 mg/d) (as shown in Fig. 2 ). The patient returned to the hospital at the age of 12 years and 3 months, exhibiting anti-nuclear antibodies (++), negative ds-DNA, anti-Smith antibodies (-), anti-ribosome antibodies (+), and anti-nucleosome antibodies (+). Blood cell analysis indicated white blood cell count 6.61 ×10^9/L, hemoglobin 124 g/L, and platelet count 254 ×10^9/L. Complement levels C3 (0.785 g/L) and C4 (0.198 g/L) were within normal range, and lymphocyte subpopulation analysis showed CD19 + 0.13% and CD20 + 0.00%. Urinary analysis showed negative results for proteinuria and red blood cells, and ESR was normal. The SLEDAI score was 0. Literature Review and Discussion The underlying pathophysiological mechanisms that contribute to the concurrent manifestation of SLE and PRCA are not well-understood. It has been postulated that the autoantibodies in SLE may either directly target bone marrow progenitor cells or interfere with erythropoietin, through either humoral or cellular immune responses [ 7 ]. Additionally, T lymphocyte dysfunction is suggested to be implicated, evidenced by the restoration of erythroid colony-forming units in the bone marrow upon T-cell depletion [ 22 ]. A thorough review of 63 documented cases detailing the co-presence of SLE and PRCA was carried out, utilizing data from Pubmed, Wanfang, and CNKI databases covering a span from 1966 to 2022. The data revealed a predominant occurrence in middle-aged and young female cohorts (Table 1 ) [ 1 – 6 , 9 – 13 , 15 – 23 , 25 – 32 , 34 – 36 , 38 – 40 , 42 – 44 , 46 ]. It was observed that PRCA often manifests subsequent to the SLE diagnosis, with around 20% of patients being diagnosed concurrently with both conditions, and a smaller portion presenting with PRCA preceding the SLE diagnosis [ 28 ]. The case under examination in our study is of particular interest as the patient developed SLE in her school-age years and maintained stability over two years post induction and maintenance therapy. Nevertheless, a relapse of her condition, marked by the onset of PRCA, ensued following the cessation of her treatment, underscoring the potential for PRCA to emerge in pediatric patients with SLE. Table 1 Literature review of the PRCA with SLE complication Year Sex Age at SLE diagnosis (years) Age at PRCA diagnosis (years) Other organs involvement Treatment Ref 2022 M 9 9 Kidney CS + CYC→CsA + Testosterone→MMF + MP pulse [1] 2021 M: 3 F: 20 31.9 ± 14.5 38.5 ± 13.2 Cutaneous, Joints, Liver CS + IVIG, HCQ, CSA, MMF, RTX, AZA, MTX, CYC [2] 2021 F 27 27 Cutaneous CS [5] 2017 M 19 19 Kidney IVIG + CS + MMF [6] 2014 F 54 47 Joint, Thyroid CS [7] 2014 F 45 55 Joint HSCT [8] 2014 F 32 52 NM CS + CYC + IVIG + rhEPO [9] 2007 NA NM NM NM CS + CsA [10] 2006 F 33 33 Cutaneous, Kidney CS + CSA [11] 2005 F 26 26 NM CS + Tripterygium glycosides [12] 2005 F 29 34 NM CS→CYC→CsA→MMF [13] 2005 F 6 6 NM NM [14] 2005 F:2 31, 31 41, 31 NM CS + EPO, CS + CSA [15] 2003 F 38 38 Cutaneous CS [16] 2003 F 42 50 NM HCQ + CS→CsA + CS [17] 2002 F 31 31 Cutaneous CS、HCQ [18] 2002 F 26 31 Cutaneous, Joint CS→AZA + plasmapheres [19] 2001 F 19 19 Joint CS [20] 2000 F 21 21 Cutaneous, Joint CsA→AZA [21] 1999 F: 2 21, 28 21, 28 Cutaneous, Muscle Liver, Thyroid IVIG、HCQ [22] 1997 F 28 28 Liver NM [23] 1996 F 69 69 Cutaneous, Joint NSAIDs、CS [24] 1995 F: 2 22.5, 40 22, 33 Cutaneous; Joint HCQ + CS; CS + CsA [4] 1995 F 50 40 Kidney, heart, lungs NSAID→AZA→CYC [25] 1995 F 32 32 NM IVIG + CS→EPO [26] 1994 M 32 43 Kideny, EPO [27] 1993 F 22 22 NM CS→IVIG [28] 1993 F 28 28 NM CS [29] 1988 M 78 78 Joints, Lung drug discontinued, rhEPO (Procainamideinduced lupus) [30] 1987 F 33 33 Joints CS [31] 1987 F 24 24 Cutaneous CS [32] 1985 F 22 22 Digest tract CS [33] 1982 F 58 58 Thyroid CS [34] 1981 F 14 14 Cutaneous, Kindney CS + CYC [35] 1978 F 56 56 Joints CS [36] 1973 F 43 53 Kidney, Joints CS [37] 1973 F 43 50 Thymoma, Endocarditisof aortic valve cusps CS [38] 1968 F 38 41 Cutaneous CS (Dead) [39] Note: AZA, Azathioprine; CS, corticosteroid; CSA, cyclosporine A; CYC, cyclophosphamide; HCQ, Hydroxychloroquine; HSCT, hematopoietic stem cell transplantation; IVIG, Intravenous immunoglobulin; MMF, Mycophenolate mofetil; MTX, methotrexate; rhEPO, Recombinant human erythropoietin; RTX, Rituximab. Literature indicates that the clinical manifestations of SLE with PRCA largely overlap with those of SLE without PRCA, though leukopenia and thrombocytopenia are more prevalent in the former. Pleurisy is less common in patients with concurrent PRCA [ 46 ], and instances of renal and central nervous system involvement, as well as serositis, appear to be infrequent [ 26 , 46 ]. Contrasting these observations, recent reports have identified renal complications characterized by significant proteinuria in this patient population [ 5 , 22 , 26 , 27 , 32 ]. A positive Coombs' test and autoimmune hemolytic anemia have also been documented in a subset of SLE with PRCA cases [ 40 , 46 ]. In the case discussed herein, the initial symptoms were dermatological and articular, with severe anemia manifesting during a relapse. Subsequent examinations confirmed PRCA and autoimmune hemolytic anemia, corroborating that the latter is a recurring complication in SLE patients with PRCA. Management of PRCA typically commences with red blood cell transfusions and extends to include treatments such as glucocorticoids, immunosuppressants, erythropoietin, and plasma exchange [ 46 ]. Prednisone is effective for most patients, and the adjunctive use of cyclosporine with glucocorticoids can enhance remission rates and diminish the risk of relapse [ 40 ]. Reports from China indicate that severe SLE can precipitate PRCA early in the disease course, with comprehensive remission achievable through combined treatment with intravenous immunoglobulin, glucocorticoids, and mycophenolate mofetil [ 27 ]. It is noteworthy that medications prescribed for SLE, including hydroxychloroquine, mycophenolate mofetil [ 3 , 8 , 45 ], and azathioprine, have been implicated in the development of PRCA, with symptoms often ameliorating upon cessation of these drugs [ 8 , 45 ]." Rituximab, a chimeric monoclonal antibody that targets CD20 on B cells [ 37 ], has been incorporated into the therapeutic regimen for systemic lupus erythematosus (SLE) for more than two decades [ 24 ]. Despite the paucity of data on its efficacy in the pediatric population [ 41 ], rituximab has demonstrated substantial effectiveness in conjunction with disease-modifying antirheumatic drugs (DMARDs) in the management of refractory lupus nephritis [ 14 ]. It also serves as a secondary treatment option for certain refractory autoimmune hematologic conditions [ 33 ]. Prior research indicates that rituximab is beneficial for SLE patients with concomitant pure red cell aplasia (PRCA) [ 28 ]. In the case detailed within this study, the patient underwent multiple transfusions of red blood cells, which initially did not yield a positive outcome. Treatment with intravenous immunoglobulin and cyclosporine, instituted due to autoimmune hemolytic anemia, did not result in clinical improvement. It was the administration of rituximab that precipitated a progressive enhancement in hemoglobin levels. The treatment plan was augmented with a sequential mycophenolate mofetil regimen, as depicted in Fig. 2 . These observations support the potential role of rituximab in treating SLE associated with PRCA, especially in scenarios where conventional treatments such as intravenous immunoglobulin, glucocorticoids, and cyclosporine are ineffective. Nonetheless, these preliminary conclusions necessitate validation through larger-scale investigations. Conclusion In conclusion, systemic lupus erythematosus (SLE) coexisting with pure red cell aplasia (PRCA) is an uncommon presentation in the pediatric population, with the majority of cases described in retrospective studies. Initial treatment strategies for SLE with concurrent PRCA generally involve glucocorticoids, with cyclosporine being added based on clinical judgment. 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Yeter KC, Afkhami M, Brynes RK,Quismorio FJ (2013) Aplastic anemia secondary to azathioprine in systemic lupus erythematosus: report of a case with normal thiopurine S-methyltransferase enzyme activity and review of the literature. Lupus 22: 1526–1528. http://doi.org/10.1177/0961203313504636 Zhang L, Zou Y, Chen Y, Zhao H, Wang S,Chen L et al. (2005) Pure red cell aplasia as presentation of systemic lupus erythematosus in children. Chinese Journal of Practical Pediatrics 20. http://doi.org/10.3969/j.issn.1005-2224.2005.07.020 Additional Declarations No competing interests reported. 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. 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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-3628283","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":251894940,"identity":"2024f2d2-5b14-4dfd-91dc-c7f62101b7a6","order_by":0,"name":"Haiyan Wang","email":"","orcid":"","institution":"Sun Yat-sen Memorial Hospital, Sun Yat-sen University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Haiyan","middleName":"","lastName":"Wang","suffix":""},{"id":251894942,"identity":"292092d7-9946-480c-a041-775827db4eaf","order_by":1,"name":"Rongqiong Ou","email":"","orcid":"","institution":"Sun Yat-sen Memorial Hospital, Sun Yat-sen University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Rongqiong","middleName":"","lastName":"Ou","suffix":""},{"id":251894944,"identity":"45a5e90a-c90b-42ab-afc1-14173b301892","order_by":2,"name":"Bihong Zhang","email":"","orcid":"","institution":"Sun Yat-sen Memorial Hospital, Sun Yat-sen University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Bihong","middleName":"","lastName":"Zhang","suffix":""},{"id":251894945,"identity":"279bee2d-7bfe-4d48-a03b-c2c321126173","order_by":3,"name":"Sha Li","email":"","orcid":"","institution":"Sun Yat-sen Memorial Hospital, Sun Yat-sen University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Sha","middleName":"","lastName":"Li","suffix":""},{"id":251894946,"identity":"1f9648c8-e8bc-4b59-ad9a-60dbea37e3a1","order_by":4,"name":"Weiping Tan","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA6klEQVRIiWNgGAWjYNCCCgYGNnY4LwG/Yh4weQaohZkkLYxtQIJoLfbsZ49JF87bJs/HzMD8mefPYQZ+9hwDhp878NjCk5cmPXPbbcM2ZgY2ad62wwySPW8MGHvP4HNYjtlt3m23GUFamHkbDjMY3MgxYAY7FZcW/jdALXNu27fBHGZPUIsEyJaG24lALQzSPGxAWyQIabnxxvz3jGO3k9uAyiTntqXzSJx5VnCwF48W9v4cY+OCmtu289ubD39488dajr89eeODn3i0gAA0RhgbmHigEXUAvwakSGT8QUjpKBgFo2AUjEgAAGCLR2m1aNxmAAAAAElFTkSuQmCC","orcid":"","institution":"Sun Yat-sen Memorial Hospital, Sun Yat-sen University","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Weiping","middleName":"","lastName":"Tan","suffix":""}],"badges":[],"createdAt":"2023-11-18 01:59:13","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3628283/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3628283/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":47076163,"identity":"a977474b-9558-4586-9661-c71ba0a6186e","added_by":"auto","created_at":"2023-11-25 23:07:59","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":1615104,"visible":true,"origin":"","legend":"\u003cp\u003eA and B are bone marrow smears, without nucleated red blood cells in the field of vision; C and D are pathology of bone marrow biopsy, with megakaryocytes in the circle of the D image. A few nucleated red blood cells are seen in the field of vision, while the rest are mostly mature granulocytes.\u003c/p\u003e","description":"","filename":"Figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-3628283/v1/691ed8a60a09876bd1709530.png"},{"id":47076161,"identity":"4da9733e-d446-4727-9dc4-2532cf125a4a","added_by":"auto","created_at":"2023-11-25 23:07:59","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":370429,"visible":true,"origin":"","legend":"\u003cp\u003eThe treatment and trend of hemoglobin after PRCA.\u003c/p\u003e\n\u003cp\u003eNote: Hb, Hemoglobin; IVIG, Intravenous immunoglobulin; MMF, Mycophenolate mofetil; Ret, Reticulocyte; RTX, Rituximab. Red arrow, infusion of concentration of red blood cells (1-2 units)\u003c/p\u003e","description":"","filename":"Figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-3628283/v1/8b0c51f40f5f094458091960.png"},{"id":47078057,"identity":"d425fd98-1e78-4944-b9f7-838572e36152","added_by":"auto","created_at":"2023-11-25 23:32:04","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2558247,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3628283/v1/f1480a06-c4d1-4079-b05c-863d92e3526e.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Pure Red Cell Aplasia Associated with Systemic Lupus Erythematosus in Children Response to Rituximab– A Case Report and Literature Review","fulltext":[{"header":"What is known","content":"\u003cp\u003eSLE-associated PRCA is a rare hematological complication that presents significant therapeutic challenges. Corticosteroids have traditionally been the mainstay of treatment for SLE-related PRCA, and the combination of cyclosporine and corticosteroids has shown promise in improving remission rates and reducing recurrence rates.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eWhat is new\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eShould the dual regimen of glucocorticoids and cyclosporine fail to elicit the desired therapeutic response, alternative approaches, such as rituximab followed by maintenance with mycophenolate mofetil, may be employed.\u003c/p\u003e"},{"header":"Introduction","content":"\u003cp\u003eSystemic Lupus Erythematosus (SLE) is a multifaceted autoimmune disorder with the potential to affect multiple organ systems. Involvement of the hematological system is frequently observed, often manifesting initially with symptoms such as anemia or immune thrombocytopenia [\u003cspan citationid=\"CR40\" class=\"CitationRef\"\u003e40\u003c/span\u003e]. Among these hematological complications, pure red cell aplastic anemia (PRCA) emerges as a particularly rare subtype of normocytic, normochromic anemia, distinguished by a deficiency in bone marrow erythropoiesis. PRCA is categorically divided into congenital and acquired forms, with the latter encompassing primary PRCA and secondary PRCA \u0026ndash; the latter of which recognizes SLE as an etiological factor [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e]. The treatment of SLE-associated PRCA, despite its clinical significance, is predominantly documented through case reports and lacks comprehensive guidelines. This study endeavors to elucidate the management of SLE-related PRCA, presenting a case report of a patient who developed PRCA subsequent to the cessation of SLE medication, complemented by a review of pertinent literature.\u003c/p\u003e"},{"header":"Materials and methods","content":"\u003cp\u003eThe clinical characteristics of the Patient were analyzed. Clinical data, including age, sex, initial symptoms, lab tests and therapy were collected. The study was conducted in accordance with the principles outlined in the Declaration of Helsinki with approval from the ethics committee of Sun Yat-sen Memorial Hospital, Sun Yat-Sen University [Ethical number: SYSKY-2023-1134-01]. Written informed consent was obtained from the Patient or her parents.\u003c/p\u003e \u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003ePatient\u0026rsquo; medical report\u003c/h2\u003e \u003cp\u003eIn August 2022, a 12-year-old girl from Maoming, Guangdong, was readmitted to our institution with the primary complaint of a three-year history of Systemic Lupus Erythematosus (SLE) and a 16-day duration of anemia. She initially presented to our hospital at 8 years and 5 months old with symptoms of skin rashes and arthralgia, her diagnostic evaluations yielded a high anti-nuclear antibody (ANA) titer of 1:1280, positive anti-double-stranded DNA (anti-ds-DNA), and positive anti-ribosomal, anti-Smith, anti-ribonucleoprotein, and anti-nucleosome antibodies. Complement levels were substantially reduced (C3: 0.225 g/L, C4: 0.031 g/L), with a complete blood count revealing leukopenia (WBC: 2.98 \u0026times;10^9/L), hemoglobin of 116 g/L, and platelets at 183 \u0026times; 10^12/L. A renal ultrasound exhibited effusions and synovial thickening in the left ankle and knee, without evidence of proteinuria or hematuria. The patient met the 2017 ACR criteria for SLE diagnosis and received treatment with cyclophosphamide (CTX) (10 mg/kg/day for two days, biweekly for a total of eight doses) and methylprednisolone (0.5 g/day for three days), which normalized the erythrocyte sedimentation rate (ESR) and 24-hour urinary protein levels. All immunologic markers including ANA, anti-ds-DNA, and complement levels returned to normal, alongside a negative urine microalbumin test. Maintenance therapy with mycophenolate mofetil was commenced, with the patient maintaining an SLE Disease Activity Index (SLEDAI) score of 0 at follow-up.\u003c/p\u003e \u003cp\u003eAt the age of 10 years and 5 months, the patient's medications, including mycophenolate mofetil, prednisone, and hydroxychloroquine, were discontinued and replaced with methotrexate. However, at 12 years of age, blood tests revealed anemia with hemoglobin levels ranging from 54 to 72 g/L and an elevated ESR (27 mm/h). Further serological examinations demonstrated positive results for anti-nuclear antibodies, anti-ds-DNA antibodies, anti-nuclear U1-nRNP antibodies, Smith antibodies, anti-nucleosome antibodies, and anti-nucleolar antibodies. Complement C3 was 0.65 g/L, and erythropoietin levels were elevated at 652.0 mIU/mL. A positive direct Coombs test led to the diagnosis of \"SLE complicated with immune hemolytic anemia.\" Intravenous immunoglobulin (1 g/kg) was administered without yielding any response. Bone marrow examination revealed active bone marrow hyperplasia with reduced proliferation of the red giant cell lineage (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eA-\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eB) and free-type homogeneous bodies accounting for 1%. Bone marrow biopsy demonstrated significant inhibition of erythroid hyperplasia (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eC-\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eD), leading to the consideration of pure red cell aplastic anemia (PRCA). Lymphocyte subgroup analysis indicated elevated proportions of CD20\u0026thinsp;+\u0026thinsp;B cells. Subsequently, the patient received methylprednisolone pulse therapy and cyclosporine, but her hemoglobin levels failed to improve, necessitating blood transfusions. Rituximab (375 mg/m^2/week \u0026times; 4 doses) was then administered, leading to gradual improvements in hemoglobin levels. Subsequent management included the sequential use of mycophenolate mofetil (20\u0026ndash;30 mg/d) (as shown in Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). The patient returned to the hospital at the age of 12 years and 3 months, exhibiting anti-nuclear antibodies (++), negative ds-DNA, anti-Smith antibodies (-), anti-ribosome antibodies (+), and anti-nucleosome antibodies (+). Blood cell analysis indicated white blood cell count 6.61 \u0026times;10^9/L, hemoglobin 124 g/L, and platelet count 254 \u0026times;10^9/L. Complement levels C3 (0.785 g/L) and C4 (0.198 g/L) were within normal range, and lymphocyte subpopulation analysis showed CD19\u0026thinsp;+\u0026thinsp;0.13% and CD20\u0026thinsp;+\u0026thinsp;0.00%. Urinary analysis showed negative results for proteinuria and red blood cells, and ESR was normal. The SLEDAI score was 0.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003eLiterature Review and Discussion\u003c/h2\u003e \u003cp\u003eThe underlying pathophysiological mechanisms that contribute to the concurrent manifestation of SLE and PRCA are not well-understood. It has been postulated that the autoantibodies in SLE may either directly target bone marrow progenitor cells or interfere with erythropoietin, through either humoral or cellular immune responses [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Additionally, T lymphocyte dysfunction is suggested to be implicated, evidenced by the restoration of erythroid colony-forming units in the bone marrow upon T-cell depletion [\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eA thorough review of 63 documented cases detailing the co-presence of SLE and PRCA was carried out, utilizing data from Pubmed, Wanfang, and CNKI databases covering a span from 1966 to 2022. The data revealed a predominant occurrence in middle-aged and young female cohorts (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e) [\u003cspan additionalcitationids=\"CR2 CR3 CR4 CR5\" citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan additionalcitationids=\"CR10 CR11 CR12\" citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e, \u003cspan additionalcitationids=\"CR16 CR17 CR18 CR19 CR20 CR21 CR22\" citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e, \u003cspan additionalcitationids=\"CR26 CR27 CR28 CR29 CR30 CR31\" citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e, \u003cspan additionalcitationids=\"CR35\" citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR36\" class=\"CitationRef\"\u003e36\u003c/span\u003e, \u003cspan additionalcitationids=\"CR39\" citationid=\"CR38\" class=\"CitationRef\"\u003e38\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR40\" class=\"CitationRef\"\u003e40\u003c/span\u003e, \u003cspan additionalcitationids=\"CR43\" citationid=\"CR42\" class=\"CitationRef\"\u003e42\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR44\" class=\"CitationRef\"\u003e44\u003c/span\u003e, \u003cspan citationid=\"CR46\" class=\"CitationRef\"\u003e46\u003c/span\u003e]. It was observed that PRCA often manifests subsequent to the SLE diagnosis, with around 20% of patients being diagnosed concurrently with both conditions, and a smaller portion presenting with PRCA preceding the SLE diagnosis [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e]. The case under examination in our study is of particular interest as the patient developed SLE in her school-age years and maintained stability over two years post induction and maintenance therapy. Nevertheless, a relapse of her condition, marked by the onset of PRCA, ensued following the cessation of her treatment, underscoring the potential for PRCA to emerge in pediatric patients with SLE.\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\u003eLiterature review of the PRCA with SLE complication\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"7\"\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 \u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eYear\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSex\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eAge at SLE diagnosis (years)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eAge at PRCA diagnosis (years)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eOther organs involvement\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTreatment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c7\"\u003e \u003cp\u003eRef\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2022\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eKidney\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;CYC\u0026rarr;CsA\u0026thinsp;+\u0026thinsp;Testosterone\u0026rarr;MMF\u0026thinsp;+\u0026thinsp;MP pulse\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[1]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2021\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eM: 3\u003c/p\u003e \u003cp\u003eF: 20\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e31.9\u0026thinsp;\u0026plusmn;\u0026thinsp;14.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e38.5\u0026thinsp;\u0026plusmn;\u0026thinsp;13.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous, Joints, Liver\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;IVIG, HCQ, CSA, MMF, RTX, AZA, MTX, CYC\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[2]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2021\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e27\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e27\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[5]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2017\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eKidney\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eIVIG\u0026thinsp;+\u0026thinsp;CS\u0026thinsp;+\u0026thinsp;MMF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[6]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2014\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e54\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e47\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eJoint, Thyroid\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[7]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2014\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e45\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e55\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eJoint\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eHSCT\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[8]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2014\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e32\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e52\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;CYC\u0026thinsp;+\u0026thinsp;IVIG\u0026thinsp;+\u0026thinsp;rhEPO\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[9]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2007\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;CsA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[10]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2006\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e33\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e33\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous, Kidney\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;CSA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[11]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2005\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e26\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e26\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;Tripterygium glycosides\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[12]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2005\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e29\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e34\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026rarr;CYC\u0026rarr;CsA\u0026rarr;MMF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[13]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2005\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[14]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2005\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF:2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e31, 31\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e41, 31\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;EPO, CS\u0026thinsp;+\u0026thinsp;CSA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[15]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2003\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e38\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e38\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[16]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2003\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e42\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eHCQ\u0026thinsp;+\u0026thinsp;CS\u0026rarr;CsA\u0026thinsp;+\u0026thinsp;CS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[17]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2002\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e31\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e31\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS、HCQ\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[18]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2002\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e26\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e31\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous, Joint\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026rarr;AZA\u0026thinsp;+\u0026thinsp;plasmapheres\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[19]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2001\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eJoint\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[20]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2000\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e21\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e21\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous, Joint\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCsA\u0026rarr;AZA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[21]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1999\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF: 2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e21, 28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e21, 28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous, Muscle\u003c/p\u003e \u003cp\u003eLiver, Thyroid\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eIVIG、HCQ\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[22]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1997\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eLiver\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[23]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1996\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e69\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e69\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous, Joint\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNSAIDs、CS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[24]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1995\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF: 2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e22.5, 40\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e22, 33\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous; Joint\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eHCQ\u0026thinsp;+\u0026thinsp;CS; CS\u0026thinsp;+\u0026thinsp;CsA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[4]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1995\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e40\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eKidney, heart, lungs\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNSAID\u0026rarr;AZA\u0026rarr;CYC\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[25]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1995\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e32\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e32\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eIVIG\u0026thinsp;+\u0026thinsp;CS\u0026rarr;EPO\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[26]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1994\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e32\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e43\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eKideny,\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eEPO\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[27]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1993\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e22\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e22\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026rarr;IVIG\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[28]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1993\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[29]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1988\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e78\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e78\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eJoints, Lung\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003edrug discontinued, rhEPO (Procainamideinduced lupus)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[30]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1987\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e33\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e33\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eJoints\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[31]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1987\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e24\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e24\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[32]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1985\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e22\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e22\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eDigest tract\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[33]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1982\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e58\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e58\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eThyroid\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[34]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1981\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e14\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e14\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous, Kindney\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u0026thinsp;+\u0026thinsp;CYC\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[35]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1978\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e56\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e56\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eJoints\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[36]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1973\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e43\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e53\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eKidney, Joints\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[37]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1973\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e43\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eThymoma, Endocarditisof aortic valve cusps\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[38]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1968\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e38\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e41\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCutaneous\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eCS (Dead)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e\u003csup\u003e[39]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"7\"\u003e\u003cem\u003eNote: AZA, Azathioprine; CS, corticosteroid; CSA, cyclosporine A; CYC, cyclophosphamide; HCQ, Hydroxychloroquine; HSCT, hematopoietic stem cell transplantation; IVIG, Intravenous immunoglobulin; MMF, Mycophenolate mofetil; MTX, methotrexate; rhEPO, Recombinant human erythropoietin; RTX, Rituximab.\u003c/em\u003e\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eLiterature indicates that the clinical manifestations of SLE with PRCA largely overlap with those of SLE without PRCA, though leukopenia and thrombocytopenia are more prevalent in the former. Pleurisy is less common in patients with concurrent PRCA [\u003cspan citationid=\"CR46\" class=\"CitationRef\"\u003e46\u003c/span\u003e], and instances of renal and central nervous system involvement, as well as serositis, appear to be infrequent [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e, \u003cspan citationid=\"CR46\" class=\"CitationRef\"\u003e46\u003c/span\u003e]. Contrasting these observations, recent reports have identified renal complications characterized by significant proteinuria in this patient population [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e, \u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e, \u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e, \u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e]. A positive Coombs' test and autoimmune hemolytic anemia have also been documented in a subset of SLE with PRCA cases [\u003cspan citationid=\"CR40\" class=\"CitationRef\"\u003e40\u003c/span\u003e, \u003cspan citationid=\"CR46\" class=\"CitationRef\"\u003e46\u003c/span\u003e]. In the case discussed herein, the initial symptoms were dermatological and articular, with severe anemia manifesting during a relapse. Subsequent examinations confirmed PRCA and autoimmune hemolytic anemia, corroborating that the latter is a recurring complication in SLE patients with PRCA.\u003c/p\u003e \u003cp\u003eManagement of PRCA typically commences with red blood cell transfusions and extends to include treatments such as glucocorticoids, immunosuppressants, erythropoietin, and plasma exchange [\u003cspan citationid=\"CR46\" class=\"CitationRef\"\u003e46\u003c/span\u003e]. Prednisone is effective for most patients, and the adjunctive use of cyclosporine with glucocorticoids can enhance remission rates and diminish the risk of relapse [\u003cspan citationid=\"CR40\" class=\"CitationRef\"\u003e40\u003c/span\u003e]. Reports from China indicate that severe SLE can precipitate PRCA early in the disease course, with comprehensive remission achievable through combined treatment with intravenous immunoglobulin, glucocorticoids, and mycophenolate mofetil [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e]. It is noteworthy that medications prescribed for SLE, including hydroxychloroquine, mycophenolate mofetil [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR45\" class=\"CitationRef\"\u003e45\u003c/span\u003e], and azathioprine, have been implicated in the development of PRCA, with symptoms often ameliorating upon cessation of these drugs [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR45\" class=\"CitationRef\"\u003e45\u003c/span\u003e].\"\u003c/p\u003e \u003cp\u003eRituximab, a chimeric monoclonal antibody that targets CD20 on B cells [\u003cspan citationid=\"CR37\" class=\"CitationRef\"\u003e37\u003c/span\u003e], has been incorporated into the therapeutic regimen for systemic lupus erythematosus (SLE) for more than two decades [\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]. Despite the paucity of data on its efficacy in the pediatric population [\u003cspan citationid=\"CR41\" class=\"CitationRef\"\u003e41\u003c/span\u003e], rituximab has demonstrated substantial effectiveness in conjunction with disease-modifying antirheumatic drugs (DMARDs) in the management of refractory lupus nephritis [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. It also serves as a secondary treatment option for certain refractory autoimmune hematologic conditions [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e]. Prior research indicates that rituximab is beneficial for SLE patients with concomitant pure red cell aplasia (PRCA) [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e]. In the case detailed within this study, the patient underwent multiple transfusions of red blood cells, which initially did not yield a positive outcome. Treatment with intravenous immunoglobulin and cyclosporine, instituted due to autoimmune hemolytic anemia, did not result in clinical improvement. It was the administration of rituximab that precipitated a progressive enhancement in hemoglobin levels. The treatment plan was augmented with a sequential mycophenolate mofetil regimen, as depicted in Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e. These observations support the potential role of rituximab in treating SLE associated with PRCA, especially in scenarios where conventional treatments such as intravenous immunoglobulin, glucocorticoids, and cyclosporine are ineffective. Nonetheless, these preliminary conclusions necessitate validation through larger-scale investigations.\u003c/p\u003e \u003c/div\u003e"},{"header":"Conclusion","content":"\u003cp\u003eIn conclusion, systemic lupus erythematosus (SLE) coexisting with pure red cell aplasia (PRCA) is an uncommon presentation in the pediatric population, with the majority of cases described in retrospective studies. Initial treatment strategies for SLE with concurrent PRCA generally involve glucocorticoids, with cyclosporine being added based on clinical judgment. Should the dual regimen of glucocorticoids and cyclosporine fail to elicit the desired therapeutic response, alternative approaches, such as rituximab followed by maintenance with mycophenolate mofetil, may be employed.\u003c/p\u003e"},{"header":"Statements and Declarations","content":"\u003cp\u003eAll authors have read the manuscript and agreed to publish in the Journal.\u0026nbsp;The study was conducted in accordance with the principles outlined in the Declaration of Helsinki with approval from the ethics committee of Sun Yat-sen Memorial Hospital, Sun Yat-Sen University [Ethical number: SYSKY-2023-1134-01]. Written informed consent was obtained from the Patient or her parents.\u003c/p\u003e\n\u003cp\u003eCompeting Interests:\u0026nbsp;The authors declare that they have no competing interests.\u003c/p\u003e\n\u003cp\u003eFunding Declaration: No Funding\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003e\u003cspan\u003e(1968) Lupus erythematosus with severe anemia, selective erythroid hypoplasia and multiple red blood cell isoantibodies. Am J Med 44: 590\u0026ndash;598. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttp://doi.org/10.1016/0002-9343(68)90059-4\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eArcasoy MO,Chao NJ (2005) T-cell-mediated pure red-cell aplasia in systemic lupus erythematosus: response to cyclosporin A and mycophenolate mofetil. 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Chinese Journal of Practical Pediatrics 20. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttp://doi.org/10.3969/j.issn.1005-2224.2005.07.020\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\n\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":"rituximab, systemic lupus erythematosus, pure red cell aplastic anemia","lastPublishedDoi":"10.21203/rs.3.rs-3628283/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3628283/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003ePurpose\u003c/strong\u003e: This study analyzes the clinical presentation of SLE-associated PRCA in a pediatric patient at our institution and evaluates rituximab's therapeutic efficacy.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMethods: \u003c/strong\u003eTo summarize the clinical characteristics of SLE related PRCA in a child diagnosed in our hospital by literature review, and discuss the efficacy of rituximab in treating this disease.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults\u003c/strong\u003e: A 12-year-old girl from Guangdong, China, was admitted to our hospital with the chief complaint of \"3 years of SLE history and anemia persisting for 16 days.\" She received her initial SLE diagnosis at 8 years and 5 months of age. Intravenous cyclophosphamide pulse and methylprednisolone (MP) pulse therapy were administered, followed by maintenance treatment with mycophenolate mofetil (MMF). During her follow-up, she maintained an SLE Disease Activity Index (SLEDAI) score of \"0.\" At 12 years of age, the patient developed progressive anemia, A bone marrow biopsy confirmed the diagnosis of pure red cell aplastic anemia. The anemia did not respond to immunoglobulin, corticosteroids, or cyclosporine A. Subsequently, rituximab was administered, leading to a gradual normalization of her hemoglobin and reticulocyte count. She was subsequently treated with MMF for maintenance, with an SLEDAI score of \"0\" during the follow-up period.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e: Corticosteroids have traditionally been the mainstay of treatment for SLE-related PRCA, and the combination of cyclosporine and corticosteroids has shown promise in improving remission rates and reducing recurrence rates. The administration of rituximab, followed by sequential MMF use in the presented case, demonstrated significant efficacy.\u003c/p\u003e","manuscriptTitle":"Pure Red Cell Aplasia Associated with Systemic Lupus Erythematosus in Children Response to Rituximab– A Case Report and Literature Review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2023-11-25 23:07:54","doi":"10.21203/rs.3.rs-3628283/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":"70ba2cd2-b1a9-4629-9ec1-e7186c2d1426","owner":[],"postedDate":"November 25th, 2023","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2023-11-25T23:07:57+00:00","versionOfRecord":[],"versionCreatedAt":"2023-11-25 23:07:54","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-3628283","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3628283","identity":"rs-3628283","version":["v1"]},"buildId":"7rjqhiLT3MXkJMwkYKINL","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}