Rheumatology最新文献

{"title":"Avacopan as an add-on therapy in a paediatric patient with new-onset granulomatosis with polyangitiis and acute kidney injury: a case report.","authors":"Robin Mørk,Pernille Bøyesen,Ingvild Andrea Kindem,Jacob Lilleby,Ragnar Gunnarsson,Øyvind Molberg,Vibke Lilleby","doi":"10.1093/rheumatology/keaf523","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf523","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"121 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145261223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tubulointerstitial inflammation driving interstitial fibrosis and tubular atrophy predicts poor renal outcome in refractory lupus nephritis","authors":"Yevgeniya Gartshteyn, Shuwei Wang, Laura Geraldino-Pardilla, Adam Mor, Vivette D D’Agati, Robert Winchester","doi":"10.1093/rheumatology/keaf530","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf530","url":null,"abstract":"OBJECTIVE Interstitial fibrosis and tubular atrophy (IF/TA) predicts ESKD in lupus nephritis (LN). We evaluated repeat kidney biopsies to identify variables driving progression of IF/TA and ESKD. METHODS LN patients with ≥2 biopsies from 1994–2018 were identified. Biopsies were interpreted by nephropathologists and classified according to ISN/RPS criteria. Clinical outcomes were ascertained through 2023. Multiplex immunohistochemistry was used to characterize the TII. RESULTS 104 LN patients (84% female, age 25 ± 12 years at diagnosis, 14% white, 40% black, 35% hispanic) with a median follow-up of 11 [6–16] years were identified. On initial biopsy, 90% had class III or IV proliferative LN with or without class V. 47 patients developed ESKD. We identified proteinuria, the presence of cellular and fibrocellular crescents, and IF/TA ≥ 25% at the first biopsy to be associated with the composite outcome of ESKD, CKD or death. In the absence of chronicity, persistent TII ≥ 25% was the only histological predictor of ESKD (OR 4.13, 95% CI 1.06, 16.06). The severity of TII on one biopsy predicted the extent of IF/TA on the subsequent biopsy. The increase in TII between the first and second biopsies was particularly high in the subgroup of patients that progressed rapidly to advanced IF/TA and ESKD. Multiplex immunohistochemistry revealed that TII could occur as tubulitis mediated by CD8 T cells or as organized T and B cell rich infiltrates reminiscent of tertiary lymphoid structures. CONCLUSION TII predicts the development of IF/TA in LN and independently from glomerulosclerosis is associated with progression to ESKD.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"87 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145260726","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diabetes mellitus and cardiac disease as key predictors of rituximab-induced acute thrombocytopenia in ANCA-associated vasculitis","authors":"Satoshi Akao, Masaru Shimizu, Kazushi Maruo, Hiromitsu Asashima, Yuya Kondo, Ayako Ohyama, Saori Abe, Ayako Kitada, Haruka Miki, Hiroto Tsuboi, Satoshi Omura, Daiki Nakagomi, Yoshiyuki Abe, Makoto Wada, Naoho Takizawa, Atsushi Nomura, Yuji Kukida, Naoya Kondo, Hirosuke Takagi, Koji Endo, Shintaro Hirata, Naoto Azuma, Tohru Takeuchi, Shoichi Fukui, Kazuro Kamada, Ryo Yanai, Yusuke Matsuo, Yasuhiro Shimojima, Ryo Nishioka, Ryota Okazaki, Tomoaki Takata, Mayuko Moriyama, Ayuko Takatani, Yoshia Miyawaki, Tsuyoshi Shirai, Hiroaki Dobashi, Takafumi Ito, Toshihiko Takada, Yutaka Kawahito, Toshiko Ito-Ihara, Takashi Kida, Nobuyuki Yajima, Takashi Kawaguchi, Isao Matsumoto","doi":"10.1093/rheumatology/keaf529","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf529","url":null,"abstract":"Objectives Rituximab (RTX) is a key treatment for ANCA-associated vasculitis (AAV), but RTX-induced acute thrombocytopenia (RIAT) remains a concern. We aimed to evaluate the risk and risk factors of RIAT in patients with AAV undergoing RTX induction therapy. Methods Patients with new-onset microscopic polyangiitis or granulomatosis with polyangiitis who received RTX in a nationwide multicentre registry in Japan were included. RIAT was defined by platelet count reductions within 28 days post-RTX. Risk factors for RIAT were identified by using logistic regression with stepwise selection, and prediction models were developed. Model performance was assessed using c-statistics. Results Among 175 patients with AAV receiving RTX, RIAT occurred in 35.4% of them. Diabetes mellitus (odds ratio [OR] = 4.96), cardiac disease (OR = 3.57), low platelet count (OR = 1.04 per 104/μL decrease), increased serum creatinine (OR = 1.44 per 1 mg/dl increase), low albumin (OR = 2.33 per 1 g/dl decrease), and high KL-6 (OR = 1.09 per 100 U/ml increase) were identified as significant predictors. A predictive model incorporating these factors achieved a C-statistic of 0.817. Conclusion RIAT is a frequent complication of RTX induction in patients with AAV, with diabetes mellitus and cardiac disease being strong risk factors. Our predictive model would enables risk assessment for RIAT, allowing clinicians to optimize treatment strategies.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"90 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145254894","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Co-occurrence of IgG4-related disease and mucous membrane pemphigoid: implication of shared IgG4-driven pathogenesis.","authors":"Koji Suzuki,Mitsuhiro Akiyama,Kanako Shimanuki,Koichi Saito,Yuko Kaneko","doi":"10.1093/rheumatology/keaf525","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf525","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"24 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145246944","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ozoralizumab in rheumatoid arthritis: a novel Fc-free anti-TNF agent with serology-independent efficacy.","authors":"Darpan R Thakare","doi":"10.1093/rheumatology/keaf526","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf526","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"14 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145246961","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The presence of synovial inflammation and response to targeted therapies.","authors":"Christopher J Edwards","doi":"10.1093/rheumatology/keaf524","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf524","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"70 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145247102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Periorbital oedema, trismus, myalgia as muscular manifestations of VEXAS syndrome: a case report and narrative literature review.","authors":"Alexis Archambeaud,Jean-Philippe Cottier,Alexandre Boissais,Kim-Lien Baud,Marie-Charlotte Besse,Benjamin Thoreau,Anne Murarasu,Nicole Ferreira,Adrien Bigot,Stéphanie Jobard,Julie Magnant,Hélène Henique,François Maillot,Alexandra Audemard-Verger","doi":"10.1093/rheumatology/keaf481","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf481","url":null,"abstract":"OBJECTIVETo report a VEXAS syndrome patient presenting with muscular manifestations, at diagnosis and to review the literature on this rare involvement.METHODSWe conducted a narrative review through 3 databases (Cochran, PubMed and Google Scholar) to identify all reports of muscle involvement associated with VEXAS syndrome. No statistical analysis was performed.RESULTSWe reported a 73-year-old male VEXAS syndrome patient presenting with muscular manifestations with ptosis, trismus, lower limb myalgia and identified 15 other patients in the literature. All were male with a median age of 71. The most common UBA1 mutations in exon 3, codon 41 involved methionine 41. Muscle inflammation at the onset of VEXAS syndrome was diagnosed by MRI or CT scan. It mostly involved orbital and facial muscles (n = 7), causing diplopia, proptosis, periorbital oedema or chemosis, and the muscles of the lower limbs, causing myalgia, weakness or oedema (n = 7). Muscle histological analysis was also performed in a few cases (n = 5) and revealed an inflammatory infiltrate with macrophages. Other symptoms were quite common: fever, skin and lung involvement, chondritis, arthralgia and thromboembolic events. Corticosteroid therapy was a routine, and the use of corticosteroid-sparing agents was almost systematic.CONCLUSIONTaken together, these new data describe the specific muscle involvement of VEXAS syndrome and extend its phenotypic spectrum. It enables us to identify three very distinct manifestations of muscle involvement: orbital, facial, and lower limb inflammation. A case series study would provide a better description of these symptoms.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"31 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145241029","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mapping a path forward: Addressing disease burden, pathways and solutions in ANCA-associated vasculitis","authors":"Bernhard Hellmich","doi":"10.1093/rheumatology/keaf517","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf517","url":null,"abstract":"Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a frequently relapsing systemic autoimmune disorder characterized by inflammation and destruction of small- to medium-sized blood vessels resulting in potentially life-threatening organ damage. Of the three AAV subtypes, granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are the most common. The aims of treatment are to rapidly control active disease with induction therapy (typically rituximab [the new standard-of-care] or cyclophosphamide alongside glucocorticoids [GC] and avacopan), followed by less aggressive maintenance strategies to reduce the risk of relapse. International and national guidelines for the treatment of GPA/MPA are generally aligned, with all guidelines highlighting a need to reduce treatment-related adverse events through rapid GC tapering and the use of GC-sparing avacopan treatment. Guidelines will continue to evolve as ongoing studies provide new insights into alternative (GC-sparing) treatment options and optimal RTX-based treatment regimens.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"77 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145228951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Understanding the burden of ANCA-associated vasculitis","authors":"Bernhard Hellmich","doi":"10.1093/rheumatology/keaf520","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf520","url":null,"abstract":"Anti-neutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a group of frequently relapsing systemic autoimmune disorders characterized by vasculitis-related organ damage and multiple comorbidities related to chronic inflammation. Advances in immunosuppression-based therapies for AAV have considerably improved remission rates, reduced the risk of relapse, and improved survival in patients with AAV. However, mortality remains high compared with the general population and the benefits of treatment are often offset by treatment-related comorbidities, organ damage and adverse effects, particularly infections. The aim of this review is to investigate the key contributors to disease burden in patients with AAV and to describe strategies for improving health-related quality of life.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"27 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145228952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"British Association of Dermatologists and British Society for Rheumatology living guideline for managing people with Behçets 2025.","authors":"Ruth Murphy, Robert J Moots, Paul Brogan, Aykut F Çelik, Mark Clement-Jones, Laura Forrest, Gülen Hatemi, Steve Higgins, Ali S M Jawad, Seema Kalra, Hilary McKee, Clare E Pain, Harry Petrushkin, Ana Poveda-Gallego, Jane F Setterfield, Poonam Sharma, Richard West, Christina Wlodek, Maria Hashme, M Firouz Mohd Mustapa, Alina M Constantin","doi":"10.1093/rheumatology/keaf521","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf521","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.4,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145213517","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}