Rheumatology最新文献

{"title":"Glucocorticoid or immunosuppressant withdrawal in SLE in remission: that is the question… the only question?","authors":"Guillermo Ruiz-Irastorza","doi":"10.1093/rheumatology/keaf331","DOIUrl":"10.1093/rheumatology/keaf331","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5204-5205"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144294862","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":"Long-term effects of selexipag in SSc-associated digital ulcers: a case-control multicentre observational study.","authors":"Claudia Iannone, Marco Di Battista, Maria Rosa Pellico, Ilaria Magi, Antonina Minniti, Giuseppe Armentaro, Silvia Cavalli, Manuel Sette, Laura Giudice, Cristina Bochicchio, Alessandra Della Rossa, Antonio G Tavoni, Fabio Cacciapaglia, Stefano Stano, Martina Orlandi, Dilia Giuggioli, Marta Mosca, Roberto Caporali, Nicoletta Del Papa","doi":"10.1093/rheumatology/keaf292","DOIUrl":"10.1093/rheumatology/keaf292","url":null,"abstract":"<p><strong>Objectives: </strong>Digital ulcers (DUs) affect ∼50% of SSc patients, causing significant pain and functional impairment. Current management involves both systemic and local therapies. However, the burden in terms of pain and quality of life due to refractory DUs still remains heavy. While selexipag is approved for SSc-associated pulmonary arterial hypertension, its potential in treating DUs is unexplored. We aimed to evaluate the long-term efficacy of selexipag compared with iloprost in treating DUs.</p><p><strong>Methods: </strong>In this multicentre case-control study, we retrospectively evaluated 96 SSc patients with refractory DUs (32 treated with selexipag, 64 with iloprost), matched for gender, disease subset and age. DU number, ischaemic pain and RP severity were assessed at baseline, 6, 12 and 24 months. Pain and RP were evaluated using the Likert Pain Scale (LPS) and Raynaud Condition Score (RCS), respectively. Additionally, DUs recurrence and new onset were recorded. Healing rates were estimated using Kaplan-Meier analysis.</p><p><strong>Results: </strong>Selexipag showed higher efficacy with 87% of DUs healing rate vs 28% for iloprost at 96 weeks (P < 0.001). DUs number, RCS and LPS showed significant improvement in selexipag-treated patients compared with iloprost (P < 0.001 for all) throughout 24 months. Selexipag-treated patients achieved faster healing (75% by week 40) and maintained significantly lower relapse rates (5% vs 45% at 24 months, P < 0.001). New DUs formation remained consistently lower in the selexipag group compared with the iloprost group (5% vs 40% at 24 months, P < 0.001).</p><p><strong>Conclusions: </strong>This observational study suggests that selexipag may be strongly effective in treating DUs refractory to conventional drugs.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5364-5371"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209395","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":"Predictive factors and clinical outcomes of progressive pulmonary fibrosis in anti-threonyl (PL7) positive anti-synthetase syndrome.","authors":"Xueyan Shan, Zhenguo Huang, Guochun Wang, Yongpeng Ge","doi":"10.1093/rheumatology/keaf306","DOIUrl":"10.1093/rheumatology/keaf306","url":null,"abstract":"<p><strong>Objectives: </strong>Patients with anti-threonyl (PL7) positive anti-synthetase syndrome (ASS) exhibit a high prevalence of interstitial lung disease (ILD), which can progress to progressive pulmonary fibrosis (PPF) and increased mortality. This study aims to explore the clinical characteristics, imaging features and predictive factors for PPF.</p><p><strong>Methods: </strong>A retrospective cohort of PL7-ASS patients at a single tertiary centre between January 2018 and December 2022 was analysed. Clinical, serological and radiological data were collected at baseline and during follow-up.</p><p><strong>Results: </strong>The study included 69 PL7-ASS patients with ILD (mean age: 54.5 years; 73.9% female). Baseline CT imaging revealed the following patterns: cellular non-specific interstitial pneumonia (cNSIP, 29%), fibrotic NSIP (fNSIP, 18.8%), organizing pneumonia (OP, 14.5%), OP-cNSIP overlap (15.9%), OP-fNSIP overlap (14.5%) and usual interstitial pneumonia (UIP, 7.2%). PPF developed in 39.1% of patients. Elevated levels of lactate dehydrogenase (LDH; HR = 1.021, 95% CI: 1.002-1.04, P = 0.031) and carbohydrate antigen 125 (CA125; HR = 1.164, 95% CI: 1.023-1.326, P = 0.022) were identified as independent predictors of PPF. Patients with UIP or OP-NSIP overlap patterns exhibited worse survival (P = 0.0479) and higher PPF prevalence (P = 0.029). Patients who developed PPF had significantly lower survival rates compared with those without PPF (HR = 5.120, 95% CI: 1.566-16.740, P = 0.018).</p><p><strong>Conclusion: </strong>PL7-ASS patients with ILD are at significant risk of developing PPF, which is associated with poor survival outcomes. Elevated LDH and CA125 levels may serve as reliable predictors of PPF, highlighting the importance of early identification and aggressive management strategies.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5379-5387"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209399","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":"High levels of EPSTI1 enhance IFN-β-mediated HLA-A expression and chemokine secretion in myoblasts in dermatomyositis.","authors":"Xiaojing Li, Ting Ding, Yizhi Xiao, Junyu Zhou, Ting Huang, Shasha Xie, Qiming Meng, Weijia He, Hongling Zhu, Hui Luo","doi":"10.1093/rheumatology/keaf297","DOIUrl":"10.1093/rheumatology/keaf297","url":null,"abstract":"<p><strong>Objectives: </strong>Epithelial-Stromal Interaction 1 (EPSTI1), an IFN-related gene that has emerged as a gene of notable interest, plays a multifaceted role in cellular function and disease processes. However, the precise role of EPSTI1 in the context of DM remains elusive and requires further exploration.</p><p><strong>Methods: </strong>To investigate EPSTI1 expression in DM, we analysed two transcriptome datasets, peripheral blood mononuclear cells (PBMCs) and muscle tissues from our DM cohort. We further validated the findings using RT-qPCR and immunohistochemical staining within our idiopathic inflammatory myopathy (IIM) cohort. Through small interfering RNA-mediated knockdown, we delved into the function and underlying mechanisms of EPSTI1 in DM patients.</p><p><strong>Results: </strong>EPSTI1 is significantly upregulated in both PBMCs and muscle tissues of DM patients. A notable positive correlation between EPSTI1 expression and IFN-stimulating genes accompanies this upregulation. Furthermore, EPSTI1 levels were markedly elevated in IFN-β-stimulated myoblasts. Functional studies have shown that downregulation of EPSTI1 inhibits multiple immune and inflammatory pathways, including antigen processing and presentation, chemokine-mediated signalling, IFN signalling, IL-1-mediated signalling, nuclear factor (NF)-κB-inducing kinase (NIK)/NF-κB signalling. EPSTI1 was involved in the expression of HLA-A and the secretion of chemokines in myoblasts mediated by IFN-β.</p><p><strong>Conclusion: </strong>EPSTI1 might play an essential role in promoting muscle inflammation in DM by regulating the expression of HLA-A and the secretion of chemokines in myoblasts. Targeting EPSTI1 may represent a novel therapeutic approach for reducing muscle inflammation and damage in dermatomyositis (DM) patients.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5529-5538"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209393","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":"Safety of biologic therapy in kidney and liver transplant recipients with systemic inflammatory diseases: a real-world study from Israel.","authors":"Victoria Furer, Omer Kersh, Mark Berman, Ayelet Grupper, Liane Rabinowich, Hagit Peleg, Elisheva Pokroy-Shapira, Ori Elkayam","doi":"10.1093/rheumatology/keaf303","DOIUrl":"10.1093/rheumatology/keaf303","url":null,"abstract":"<p><strong>Objectives: </strong>Safety is a concern for solid-organ transplant (SOT) recipients with systemic inflammatory diseases (SIDs) treated with biologic therapy. This study evaluated the safety of biologic therapy in SOT recipients with SIDs.</p><p><strong>Methods: </strong>This retrospective study between 2000 and 2024 included 20 biologic-treated SOT recipients with SIDs matched to 56 SOT control recipients without SIDs, not treated with biologic therapy. The study compared post-transplant safety outcomes, with serious infections defined as the primary outcome. Kaplan-Meier survival analysis evaluated time-to-safety event outcomes.</p><p><strong>Results: </strong>The biologic-treated group included patients with SIDs, mainly with IBD and FMF treated with TNF and IL-1 inhibitors, respectively, 60% (n = 12) of whom were treated with biologics over 5 years post-SOT. There was a non-significant trend for serious infections in the biologic-treated group vs controls, 40% (n = 8) vs 23.21% (n = 13), P = 0.15, with urinary tract infections being the most prevalent in both groups. Recurrent serious infections were more prevalent in the biologic-treated group vs controls, 20% (n = 4) vs 12.5% (n = 2), P = 0.029. No opportunistic infections were observed. No graft rejection occurred in the biologic-treated group, compared with 5.37% in the control group. Cancer rates in the biologic-treated group were comparable with those in the control group, 15% vs 7.14%, P = 0.3. There were two deaths in the biologic-treated group (one attributed to COVID-19 infection and one from a car accident), compared with none in the control group, P = 0.003. There were six cases of biologic treatment discontinuation, mainly due to loss of efficacy.</p><p><strong>Conclusion: </strong>This real-world cohort with 5-year follow-up since the post-SOT biologic initiation supported the feasibility of biologic therapy in SOT recipients with SIDs.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5372-5378"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209400","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":"Ruxolitinib for refractory lung granulomatosis in two paediatric patients.","authors":"Marine Michelet, Benjamin Fournier, Benjamin Terrier, Dominique Valeyre, Jean-François Bernaudin, Cécile Lozach, Karine Brochard, Naziha Khen-Dunlop, Christophe Delacourt, Laureline Berteloot, Anna Vigier, Dominique Berrebi, Brigitte Bader-Meunier, David Drummond","doi":"10.1093/rheumatology/keaf333","DOIUrl":"10.1093/rheumatology/keaf333","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5573-5575"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275764","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":"Avacopan may not independently reduce ANCA titres in ANCA-associated vasculitis: a retrospective analysis of 57 patients.","authors":"Motoki Takeuchi, Yoshiyuki Abe, Masahiro Kogami, Ayako Makiyama, Ken Yamaji, Naoto Tamura","doi":"10.1093/rheumatology/keaf339","DOIUrl":"10.1093/rheumatology/keaf339","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5564-5566"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144294861","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":"Comment on: Risk factors of digital gangrene secondary to systemic lupus erythematosus flare: real-world data from 2014 to 2022: reply.","authors":"Yang Liu, Qian Li, Pengyan Qiao, Ying Liu, Wenqin Gao, Yanli Yang, Sumiao Liu, Ke Xu","doi":"10.1093/rheumatology/keaf379","DOIUrl":"10.1093/rheumatology/keaf379","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5580"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144660069","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":"DISH as a marker for incident diabetes mellitus in cardiovascular disease patients.","authors":"Netanja I Harlianto, Wouter Foppen, Firdaus A A Mohamed Hoesein, Marjolein E Hol, Jorrit-Jan Verlaan, Pim A de Jong, Jan Westerink","doi":"10.1093/rheumatology/keaf268","DOIUrl":"10.1093/rheumatology/keaf268","url":null,"abstract":"<p><strong>Objectives: </strong>DISH is a common incidental finding in medical imaging characterized by continuous vertebral ossification, which is associated with prevalent type 2 diabetes mellitus (T2DM). We hypothesized that incidental screen-detected DISH may be an actionable marker for incident diabetes screening and aimed to assess the absolute incidence rate (ratio) for T2DM in cardiovascular patients with and without DISH.</p><p><strong>Methods: </strong>Cardiovascular disease patients without diabetes (n = 3395) were included via the prospective Second Manifestation of ARTerial disease cohort. DISH was evaluated at baseline on chest radiographs using Resnick criteria. Incident T2DM was assessed by an adjudication committee. Adjusted incidence rate ratios (IRRs) and numbers needed to screen were calculated.</p><p><strong>Results: </strong>DISH was present in 263 (7.7%) patients. After a median follow-up of 11.1 years (IQR: 7.1-15.2), 317 patients developed T2DM. Patients with DISH had a higher incidence rate for T2DM compared with no-DISH patients (17.1 vs. 7.8 T2DM per 1000 person-years). DISH was associated with incident T2DM in multivariate analyses (IRR: 1.47; 95% CI: 1.03-2.06), with the highest IRR in the DISH group with the most extensive ossification (IRR: 2.01; 95% CI: 1.15-3.29). The number needed to screen for T2DM in patients with screen-detected DISH for 11.1 years was 7, similar to accepted risk markers overweight (n = 8), obesity (n = 5), hypertension (n = 9) and hyperlipidaemia (n = 13).</p><p><strong>Conclusions: </strong>DISH is associated with higher rates of incident T2DM in cardiovascular disease patients, independent of accepted risk markers. DISH could be used as an imaging marker to identify cardiovascular disease patients with an increased risk for subsequent T2DM.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5287-5294"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12494210/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144161773","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lung function and skin fibrosis changes as predictors of survival in SSc-associated interstitial lung disease: a EUSTAR study.","authors":"Vincent Sobanski, Jeska de Vries-Bouwstra, Anna-Maria Hoffmann-Vold, Dörte Huscher, Margarida Alves, Marco Matucci-Cerinic, Gabriela Riemekasten, Mengtao Li, László Czirják, Otylia Kowal-Bielecka, Yannick Allanore, Nils Schoof, Oliver Distler","doi":"10.1093/rheumatology/keaf264","DOIUrl":"10.1093/rheumatology/keaf264","url":null,"abstract":"<p><strong>Objectives: </strong>This study assessed how changes in lung function, skin fibrosis and digital ulceration (DU) burden predict mortality in patients with SSc-associated interstitial lung disease (SSc-ILD), the leading cause of death in SSc.</p><p><strong>Methods: </strong>Adult SSc-ILD patients from the European Scleroderma Trials and Research (EUSTAR) database enrolled since January 2009 with a date of diagnosis, a follow-up visit for change evaluation within 12 months plus a further visit or mortality information were eligible. Twelve-month changes in lung function (per cent predicted forced vital capacity [FVC%pred] and diffusing capacity of the lungs for carbon monoxide [DLCO%pred]), modified Rodnan skin score (mRSS) and change in DU burden were assessed for associations with survival, using multivariable Cox regression analyses adjusted for age, sex, smoking status and immunosuppressive therapy.</p><p><strong>Results: </strong>Of 893 SSc-ILD patients included, 94 (10.5%) died over a mean follow-up of 39.0 ± 23.9 months. Absolute deterioration in FVC >10%pred within 12 months (n = 78/638 evaluable) was predictive for decreased survival (hazard ratio [HR] 3.81; 95% CI 1.67-8.66), as were composite measures combining (i) >10% FVC decline or mRSS worsening (HR 2.82; 95% CI 1.43-5.56) and (ii) FVC decline ≥10% or 5-9% with DLCO decline ≥15% (HR 3.42; 95% CI 1.68-7.00), but not changes in DLCO, mRSS or DU burden alone.</p><p><strong>Conclusions: </strong>Changes in lung function and skin fibrosis within 12 months should be considered when evaluating risk of mortality. The effect of pharmacological treatments aiming at stabilization of these variables should be evaluated prospectively in clinical trials.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"5344-5353"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12494203/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}