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Drug retention rate of Janus kinase inhibitors in patients with rheumatoid arthritis: the KEIO-RA cohort study. 类风湿关节炎患者Janus激酶抑制剂的药物保留率:KEIO-RA队列研究
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-11-24 DOI: 10.1080/25785826.2025.2590230
Satoshi Takanashi, Masatoshi Kawai, Mitsuhiro Akiyama, Yasushi Kondo, Shuntaro Saito, Masaru Takeshita, Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko
{"title":"Drug retention rate of Janus kinase inhibitors in patients with rheumatoid arthritis: the KEIO-RA cohort study.","authors":"Satoshi Takanashi, Masatoshi Kawai, Mitsuhiro Akiyama, Yasushi Kondo, Shuntaro Saito, Masaru Takeshita, Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko","doi":"10.1080/25785826.2025.2590230","DOIUrl":"10.1080/25785826.2025.2590230","url":null,"abstract":"<p><p>This study aimed to investigate the effectiveness and safety of Janus kinase inhibitors (JAKis) in patients with rheumatoid arthritis (RA) in the real world. All consecutive patients with RA from the KEIO-RA cohort who had received a JAKi from July 2013 to June 2023 were included. We assessed drug retention rates, factors associated with retention, disease activity, and adverse events. In total, 294 treatment courses in 201 patients were analyzed. The mean drug continuation duration was 17.2 ± 17.4 months. Full-dose JAKi use and concomitant methotrexate (MTX) use were associated with higher retention rates (<i>p</i> < 0.001 and <i>p</i> = 0.03, respectively). Combination therapy with full-dose JAKi and MTX showed a significantly lower discontinuation rate due to lack of efficacy (<i>p</i> = 0.02), with no increase in safety issues. This pattern was observed across age groups and difficult-to-treat status, though residual confounding cannot be excluded. When comparing retention across five JAKi formulations among patients receiving full-dose JAKi with concomitant MTX, upadacitinib and filgotinib showed significantly lower discontinuation rates due to lack of efficacy (<i>p</i> = 0.04), whereas baricitinib had a lower discontinuation rate due to safety issues (<i>p</i> = 0.01). In conclusion, combination therapy with full-dose JAKi and MTX showed higher retention in this cohort.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"188-200"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145589204","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pustulotic arthro-osteitis with distal phalanx osteitis. 脓疱性关节骨炎伴远端指骨骨炎。
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-10-21 DOI: 10.1080/25785826.2025.2577518
Takuma Koga, Shinjiro Kaieda, Makiko Ebuchi, Kazuhide Shimamatsu, Hiroaki Ida, Tomoaki Hoshino
{"title":"Pustulotic arthro-osteitis with distal phalanx osteitis.","authors":"Takuma Koga, Shinjiro Kaieda, Makiko Ebuchi, Kazuhide Shimamatsu, Hiroaki Ida, Tomoaki Hoshino","doi":"10.1080/25785826.2025.2577518","DOIUrl":"10.1080/25785826.2025.2577518","url":null,"abstract":"<p><p>A 68-year-old Japanese woman was diagnosed with palmoplantar pustulosis. She presented with anterior chest pain and swelling, as well as pain in the right first finger. Initially, antibiotics was ineffective, the patient was diagnosed with non-bacterial osteomyelitis. Computed tomography of the chest revealed a sternal bone thickening pattern, and technetium-99m bone scintigraphy showed accumulations in the left sternoclavicular joint and sternal angle. Additionally, contrast-enhanced magnetic resonance imaging revealed osteitis in the sternal scapes and body. Based on these findings, the patient was diagnosed with pustulotic arthro-osteitis. The swelling of the right first finger repeatedly worsened and resolved spontaneously, the first right distal phalanx was amputated to rule out infection and malignancy. Histopathological evaluation revealed evidence of chronic non-bacterial osteitis, and associated with pustulotic arthro-osteitis. We report this case as histopathological examination is rarely performed to confirm the diagnosis of finger osteitis associated with pustulotic arthro-osteitis.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"253-259"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145337696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Identification of BHLHE40-expressing T cells in giant cell arteritis amplified by interleukin-1. 白细胞介素-1扩增巨细胞动脉炎中表达bhlhe40的T细胞的鉴定
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-12-03 DOI: 10.1080/25785826.2025.2594237
Sho Ishigaki, Katsuya Suzuki, Kazuhiro Yamanoi, Takashi Nishina, Yoichiro Iwakura, Yuko Kaneko
{"title":"Identification of BHLHE40-expressing T cells in giant cell arteritis amplified by interleukin-1.","authors":"Sho Ishigaki, Katsuya Suzuki, Kazuhiro Yamanoi, Takashi Nishina, Yoichiro Iwakura, Yuko Kaneko","doi":"10.1080/25785826.2025.2594237","DOIUrl":"10.1080/25785826.2025.2594237","url":null,"abstract":"<p><p>BHLHE40 is a transcription factor regulating proinflammatory cytokines such as granulocyte-macrophage colony-stimulating factor (GM-CSF) in CD4<sup>+</sup> T cells. Although implicated in autoimmune inflammation, its regulation by interleukin (IL)-1 signaling in human CD4<sup>+</sup> T cells remains unclear. Peripheral blood (PB) from healthy controls (HCs) and patients with giant cell arteritis (GCA) was analyzed using flow cytometry to assess BHLHE40 expression across helper T cells. Immunohistochemistry was performed on the aortas of IL-1 receptor antagonist knockout (IL-1Rn KO) mice and temporal artery biopsies from GCA patients. We also analyzed public microarray datasets and CD4<sup>+</sup> T cells stimulated with anti-CD3/CD28 and IL-1β. We identified BHLHE40 expression in T cells in the IL-1Rn KO mice and GCA-inflamed arteries. BHLHE40 expression was higher in helper T subsets than in naïve CD4<sup>+</sup> T cells. Co-stimulation with IL-1β and anti-CD3/CD28 induced stronger BHLHE40 expression than CD3/CD28 alone. Microarray data showed increased expression of <i>BHLHE40</i> in CD4<sup>+</sup> T cells from the PB of GCA patients. IL-1 signaling enhances BHLHE40 expression during CD4<sup>+</sup> T cell activation. Its sustained expression in inflamed tissues suggests a disease-relevant pathway linking IL-1 signaling to pathogenic T cell responses in GCA.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"201-211"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145670194","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The role of immunoglobulin E in upper respiratory allergies: mechanisms and therapeutic strategies. 免疫球蛋白E在上呼吸道过敏中的作用:机制和治疗策略。
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-11-21 DOI: 10.1080/25785826.2025.2587474
Shiji Li, Jingyun Li, Yuan Zhang, Luo Zhang
{"title":"The role of immunoglobulin E in upper respiratory allergies: mechanisms and therapeutic strategies.","authors":"Shiji Li, Jingyun Li, Yuan Zhang, Luo Zhang","doi":"10.1080/25785826.2025.2587474","DOIUrl":"10.1080/25785826.2025.2587474","url":null,"abstract":"<p><p>This review focuses on the central role of Immunoglobulin E (IgE) in upper respiratory allergic diseases and associated therapeutic strategies. As the core immunoglobulin driving type 2 inflammation, IgE production is meticulously regulated by multiple cytokines and signaling pathways. Current therapeutic approaches primarily involve two strategies: direct inhibition of IgE generation and function, and promotion of Immunoglobulin G4 (IgG4) and Immunoglobulin A (IgA) production to competitively suppress IgE-mediated responses.For direct IgE inhibition, monoclonal antibodies such as omalizumab and dupilumab exert their effects by directly antagonizing IgE or blocking upstream inflammatory cytokines critical for IgE synthesis. A spectrum of other multitarget monoclonal antibodies and non-antibody biologics are also under active investigation. Allergen immunotherapy (AIT) represents the principal approach for enhancing IgG4 and IgA production, aiming to rebalance immune responses through modulation of cytokine profiles, induction of regulatory T cells, and augmentation of allergen-specific IgG4/IgA titres to neutralize allergens and attenuate type 2 inflammation. This article comprehensively dissects the molecular mechanisms underlying IgE biosynthesis, pathological roles in allergic inflammation, and therapeutic intervention strategies, thereby providing novel insights for optimizing treatment protocols and improving clinical outcomes in upper respiratory allergic disorders.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"147-163"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145574803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Organ damage accrual in patients with newly onset systemic lupus erythematosus with neuropsychiatric symptoms and its clinical features in daily clinical practice: a single-center study. 新发系统性红斑狼疮伴神经精神症状的器官损害及其临床特征:一项单中心研究
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-12-09 DOI: 10.1080/25785826.2025.2597049
Kenji Saito, Fumitaka Seike, Shuzo Sato, Shotaro Ogawa, Yuya Sumichika, Shuhei Yoshida, Haruki Matsumoto, Jumpei Temmoku, Yuya Fujita, Tomoyuki Asano
{"title":"Organ damage accrual in patients with newly onset systemic lupus erythematosus with neuropsychiatric symptoms and its clinical features in daily clinical practice: a single-center study.","authors":"Kenji Saito, Fumitaka Seike, Shuzo Sato, Shotaro Ogawa, Yuya Sumichika, Shuhei Yoshida, Haruki Matsumoto, Jumpei Temmoku, Yuya Fujita, Tomoyuki Asano","doi":"10.1080/25785826.2025.2597049","DOIUrl":"10.1080/25785826.2025.2597049","url":null,"abstract":"<p><p>This study was conducted to reveal specific clinical features, outcomes and risk factors of organ damage in patients with systemic lupus erythematosus (SLE) with neuropsychiatric (NP) symptoms upon initial onset. Patients with newly diagnosed SLE at our institute between 2010 and 2022 were enrolled. Patients who exhibited NP symptoms at initial onset constituted the initial onset NPSLE (Ini-NPSLE) group, and their clinical features were compared with those of patients without NP symptoms (SLE NP- group). Among 90 patients with newly onset SLE (mean age 37.7 years), there were 20 patients in the Ini-NPSLE group and 70 patients in the SLE NP- group. General clinical characteristics were similar between the two groups, except for diagnostic delay, arthritis and avascular necrosis. The Ini-NPSLE group showed significantly higher SLE Disease Activity Index scores at admission (24.8 vs. 15.6) and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) scores (1.55 vs. 0.53) at the end of follow-up. Logistic regression analysis revealed that patients' age and NPSLE were associated with organ damage (SDI ≥ 1) in patients with newly onset SLE. This study suggests that clinicians must focus on early NP symptoms to manage later organ damage in patients with SLE.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"223-231"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145716179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Successful treatment of tofacitinib-refractory scleritis associated with multiple systemic inflammatory diseases using upadacitinib. 使用upadacitinib成功治疗托法西替尼难治性巩膜炎与多发性全身性炎症疾病。
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-11-20 DOI: 10.1080/25785826.2025.2588918
Kodai Yuge, Nobuyo Yawata, Kenichiro Asahara, Satoshi Yamana, Nobuyuki Ono, Koh-Hei Sonoda
{"title":"Successful treatment of tofacitinib-refractory scleritis associated with multiple systemic inflammatory diseases using upadacitinib.","authors":"Kodai Yuge, Nobuyo Yawata, Kenichiro Asahara, Satoshi Yamana, Nobuyuki Ono, Koh-Hei Sonoda","doi":"10.1080/25785826.2025.2588918","DOIUrl":"10.1080/25785826.2025.2588918","url":null,"abstract":"<p><p>Scleritis is often associated with systemic inflammatory diseases, and a subset of patients remains refractory to conventional therapies, including corticosteroids, immunosuppressants, and tumor necrosis factor inhibitors (TNFi). Janus kinase inhibitors (JAKi) exert anti-inflammatory effects by broadly suppressing downstream cytokine signaling and have been increasingly applied in the management of systemic inflammatory conditions. Recently, more selective JAKi have been developed to minimize adverse effects while preserving therapeutic efficacy. However, clinical experience comparing the efficacy of non-selective versus selective JAKi remains limited. We report a case of highly refractory scleritis positive for proteinase 3-antineutrophil cytoplasmic antibody and suffered from rheumatoid arthritis (RA), and ulcerative colitis (UC). While RA and UC were controlled with TNFi, scleritis remained active and difficult to manage. Following the initiation of tofacitinib, a pan-JAKi, the scleritis rapidly entered remission. However, after tapering and discontinuation of methotrexate and corticosteroids, the scleritis relapsed, suggesting that control was not maintained with tofacitinib and became refractory to treatment. A subsequent switch to upadacitinib, a selective JAK1 inhibitor, resulted in renewed remission and sustained control of ocular inflammation. This case highlights the potential of JAKi as a treatment option for refractory scleritis, particularly in patients unresponsive to conventional therapies, and the possibility of transitioning to a selective JAK1 inhibitor in cases of pan-JAKi resistance.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"266-274"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145565801","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction. 修正。
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-12-22 DOI: 10.1080/25785826.2025.2605862
{"title":"Correction.","authors":"","doi":"10.1080/25785826.2025.2605862","DOIUrl":"10.1080/25785826.2025.2605862","url":null,"abstract":"","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"I"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145811548","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Rozanolixizumab as rescue therapy in triple-seronegative refractory generalized myasthenia gravis. 罗扎诺单抗作为三血清阴性难治性全身性重症肌无力的抢救治疗。
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2025-11-10 DOI: 10.1080/25785826.2025.2582274
Yohei Takenobu, Takayuki Kikuchi, Daiki Ishikawa, Noriko Nomura, Shunsuke Tamaki, Kayoko Yukawa, Mari Inoue, Karen Hatano, Junichi Miyahara, Kentaro Yamakawa, Osamu Higuchi, Manabu Inoue
{"title":"Rozanolixizumab as rescue therapy in triple-seronegative refractory generalized myasthenia gravis.","authors":"Yohei Takenobu, Takayuki Kikuchi, Daiki Ishikawa, Noriko Nomura, Shunsuke Tamaki, Kayoko Yukawa, Mari Inoue, Karen Hatano, Junichi Miyahara, Kentaro Yamakawa, Osamu Higuchi, Manabu Inoue","doi":"10.1080/25785826.2025.2582274","DOIUrl":"10.1080/25785826.2025.2582274","url":null,"abstract":"<p><p>Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, most commonly associated with anti-acetylcholine receptor antibodies, less frequently with muscle-specific kinase antibodies, and occasionally with low-density lipoprotein receptor-related protein 4 antibodies. However, a subset of patients with MG tests negative for all of these three antibodies - termed triple-seronegative MG - posing significant diagnostic and therapeutic challenges. Fast-acting therapies, such as intravenous methylprednisolone (IVMP), intravenous immunoglobulin (IVIG) and plasma exchange (PLEX), are typically recommended during disease exacerbation. However, some patients are refractory to these interventions. For refractory patients with triple-seronegative MG, the efficacy of a recent monoclonal antibody therapy as rescue therapy remains uncertain. We report a case of triple-seronegative MG refractory to repeated IVIG, IVMP and PLEX, in which clinical improvement was achieved following treatment with rozanolixizumab. This case, with long-term follow-up, suggests the potential utility of rozanolixizumab as rescue therapy in a patient subgroup which is typically excluded from randomized controlled trials. Larger studies specifically including patients with triple-seronegative MG are warranted.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"260-265"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145483283","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Questionnaire-based nationwide survey on the safety of azathioprine in Japanese patients with rheumatic diseases: a cross-sectional study. 日本风湿病患者使用硫唑嘌呤安全性的问卷调查:横断面研究。
IF 2.9
Immunological Medicine Pub Date : 2026-06-01 Epub Date: 2026-01-02 DOI: 10.1080/25785826.2025.2604344
Shuhei Yoshida, Kiyoshi Migita, Tomoyuki Asano, Haruki Matsumoto, Shuzo Sato, Eiji Suzuki, Tomomi Sasajima, Masayuki Miyata, Michio Onizawa, Kazumichi Abe, Atsushi Takahashi, Hiromasa Ohira
{"title":"Questionnaire-based nationwide survey on the safety of azathioprine in Japanese patients with rheumatic diseases: a cross-sectional study.","authors":"Shuhei Yoshida, Kiyoshi Migita, Tomoyuki Asano, Haruki Matsumoto, Shuzo Sato, Eiji Suzuki, Tomomi Sasajima, Masayuki Miyata, Michio Onizawa, Kazumichi Abe, Atsushi Takahashi, Hiromasa Ohira","doi":"10.1080/25785826.2025.2604344","DOIUrl":"10.1080/25785826.2025.2604344","url":null,"abstract":"<p><p>Azathioprine (AZA) is an established treatment for rheumatic diseases, but real-world safety data in Japanese patients remain limited. This study aimed to evaluate the real-world use and safety of AZA in Japan. A nationwide questionnaire survey was conducted, distributing forms to 1163 facilities, including university hospitals and Japan College of Rheumatology-certified institutions. Of these, 170 facilities (14.6%) responded. A total of 1943 patients with rheumatic diseases who initiated AZA between November 2000 and September 2023 were enrolled. Among them, 33.9% experienced adverse events (AEs), including hepatobiliary disorders (13.9%), gastrointestinal disorders (10.4%), blood and lymphatic system disorders (9.3%), infections (5.1%), and skin/subcutaneous disorders (2.4%). AZA therapy was discontinued in 468 (71.6%) of the 660 patients with AEs. The incidence of serious AEs (grade ≥3) was 2.7% and varied by rheumatic disease. Multivariate analysis identified older age (odds ratio [OR] 2.47, 95% confidence interval [CI]: 1.32-4.59) and systemic lupus erythematosus (OR 2.31, 95% CI: 1.09-4.87) as risk factors for serious AEs. This nationwide survey provides evidence of AZA-related AEs in Japanese patients with rheumatic diseases. AE incidence was relatively high, and serious AEs (grade ≥3) occurred more frequently in patients aged over 65 years and those with systemic lupus erythematosus.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"232-243"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145893292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Systemic sclerosis with concurrent ANCA-positive IgA nephropathy successfully managed with rituximab and mycophenolate mofetil: a case report. 利妥昔单抗和霉酚酸酯成功治疗系统性硬化症并发anca阳性IgA肾病1例报告。
IF 2.9
Immunological Medicine Pub Date : 2026-05-16 DOI: 10.1080/25785826.2026.2671471
So Yamamoto, Shuhei Yoshida, Isamu Higuchi, Kenji Saito, Jumpei Temmoku, Tasuku Ishikawa, Shuhei Watanabe, Akihito Utsumi, Haruki Matsumoto, Tomoyuki Asano, Shuzo Sato, Yuko Hashimoto, Yasuhiro Shimojima
{"title":"Systemic sclerosis with concurrent ANCA-positive IgA nephropathy successfully managed with rituximab and mycophenolate mofetil: a case report.","authors":"So Yamamoto, Shuhei Yoshida, Isamu Higuchi, Kenji Saito, Jumpei Temmoku, Tasuku Ishikawa, Shuhei Watanabe, Akihito Utsumi, Haruki Matsumoto, Tomoyuki Asano, Shuzo Sato, Yuko Hashimoto, Yasuhiro Shimojima","doi":"10.1080/25785826.2026.2671471","DOIUrl":"https://doi.org/10.1080/25785826.2026.2671471","url":null,"abstract":"<p><p>Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy and fibrosis, whereas antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small-vessel vasculitis that frequently involves the kidneys and lungs. Although an overlap between SSc and AAV has been reported, ANCA-positive IgA nephropathy (IgAN) in SSc has not been described. Clinically, ANCA-positive IgAN resembles AAV, posing challenges for disease classification and management. However, the optimal treatment strategy for such overlapping cases has not yet been established. We report the case of a 73-year-old woman with long-standing SSc and interstitial lung disease who developed rapidly progressive glomerulonephritis. She was positive for myeloperoxidase-ANCA, and renal biopsy demonstrated crescentic glomerulonephritis with mesangial IgA and C3 deposition, raising the possibility of concomitant IgAN. The patient was treated with glucocorticoids (GCs) and rituximab (RTX) as induction therapy, followed by mycophenolate mofetil (MMF) as maintenance therapy, resulting in a marked reduction in proteinuria and stabilization of renal function. This case underscores the importance of considering ANCA-positive IgAN in SSc patients presenting with renal dysfunction and suggests that combination therapy with RTX and MMF may represent a feasible therapeutic option for this rare overlap condition.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-8"},"PeriodicalIF":2.9,"publicationDate":"2026-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147960481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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