Rheumatology最新文献

{"title":"Increased mortality rate in patients with Takayasu arteritis is largely driven by early disease activity and damage: analysis of two cohorts.","authors":"Swapnil Jagtap, Aysegul Avcu, Sandeep Balakrishnan, Manas Ranjan Behera, Neeraj Jain, Anuradha Singh, Manish Ora, Roopali Khanna, Haner Direskeneli, Fatma Alibaz-Oner, Durga Prasanna Misra","doi":"10.1093/rheumatology/keag540","DOIUrl":"10.1093/rheumatology/keag540","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate mortality risk and pre-treatment predictors of mortality in Takayasu arteritis (TAK).</p><p><strong>Methods: </strong>From two cohorts, outcomes at last visit (survival/mortality) were recorded. Standardized mortality ratios (SMR) were computed (indirect standardization to population death rates from India and Turkiye). Cox proportional hazards regression was used to estimate predictors of mortality [hazard ratios (HR) with 95%CI]. The final multivariable-adjusted models included significant predictors of mortality from initial analyses based on demographic features, disease activity, damage (VDI) and clinical features or arterial involvement. Random forest (RF) and gradient boosted machines (GBM) machine learning tree models were used to understand the relative contributions of covariates in the multivariable-adjusted regression models to mortality risk.</p><p><strong>Results: </strong>Among 529 patients with TAK [India, n = 310, Turkiye, n = 219, mean age at onset 29.12 years, 413 females], 54 deaths were recorded (29 out-of-hospital, 29/54 from cardiovascular disease, 10/54 from infections). Survival was 97.73%, 96.55%, 90.24% and 82.37% at 1, 2, 5 and 10 years, respectively. TAK was associated with increased mortality rate [SMR for India 35.88 (95%CI 24.41-50.94) and Turkiye 27.61 (95%CI 17.53-41.41)]. Higher baseline disease activity by ITAS2010 (HR 1.06), damage (HR 1.30), impaired renal function (HR 2.21) or abdominal aorta involvement (HR 2.03) significantly predicted mortality risk with good model discrimination accuracy (Harrell's C-statistic 0.772). Both RF and GBM models identified baseline activity and damage scores as major drivers of mortality. New-onset vascular events were largely driven by baseline ITAS2010.</p><p><strong>Conclusion: </strong>Increased mortality rate in TAK is largely driven by disease activity and early damage.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149040468","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-dose short-interval canakinumab therapy for mevalonate kinase deficiency associated with novel\u0000 MVK\u0000 variants","authors":"Yoshitake Sato,Daigo Kato,Kanako Mitsunaga,Yuzaburo Inoue,Minako Tomiita","doi":"10.1093/rheumatology/keag548","DOIUrl":"https://doi.org/10.1093/rheumatology/keag548","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"1 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149089288","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":"Obesity is more strongly associated with physical performance and body composition than sarcopenia in patients with spondyloarthritis","authors":"Uta Kiltz,Julia Kononenko,Marius Bedei,Imke Redeker,David Kiefer,Philipp Sewerin,Ioana Andreica,Anna Kernder,Diana Vossen,Bjoern Bühring,Ralph Brinks,Xenofon Baraliakos","doi":"10.1093/rheumatology/keag530","DOIUrl":"https://doi.org/10.1093/rheumatology/keag530","url":null,"abstract":"Abstract Objectives Integrated data on body composition, objective physical performance and daily physical activity are limited in spondyloarthritis (SpA). The objective of this study is to describe DXA-derived body composition phenotypes and examine their cross-sectional associations with objective physical performance and daily physical activity in axial SpA (axSpA) and psoriatic arthritis (PsA). Methods In this single-center cross-sectional study, 213 patients with axSpA or PsA underwent performance testing and completed the mSQUASH questionnaire. Obesity was defined using DXA-derived body fat percentage (≥30% in men, ≥40% in women). Physical activity was categorized into tertiles. ANCOVA models adjusted for age, BMI and diagnosis assessed independent effects of physical activity on performance and body composition. Results Physical performance was largely preserved. DXA-defined obesity was common whereas sarcopenia was rare. Higher levels of daily activity were consistently associated with better physical performance and favorable body composition. Patients in the highest mSQUASH activity tertile demonstrated significantly higher grip strength (45.3 (13.0) kg), faster gait speed (1.60 (0.27) m/s), and higher SPPB scores (11.5 (1.0), alongside lower FMI and higher ALM/BMI. In sex-stratified analyses, TNFi-treated men had lower body fat and obesity prevalence than men on conventional therapy; no differences were observed in women. In adjusted models, daily physical activity remained independently associated with ALM/BMI, grip strength and ASPI, whereas diagnosis showed no independent association. Conclusion In SpA, DXA-defined obesity was common and associated with worse objective performance, whereas sarcopenia was rare. Higher levels of daily physical activity were independently associated with better physical performance and more favourable muscle-related body composition indices.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"52 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149049131","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":"Oral microbiota dysbiosis in Sjögren’s disease occurs independent of hyposalivation","authors":"Diana Estefania Ramos Peña,Daira Ester de Sousa,Fabíola Reis de Oliveira,Eduardo Melani Rocha,Alan Grupioni Lourenço,Ana Carolina F Motta","doi":"10.1093/rheumatology/keag538","DOIUrl":"https://doi.org/10.1093/rheumatology/keag538","url":null,"abstract":"Abstract Objectives The objective of this study was to compare the oral microbiota of patients with SjD and controls, and to correlate these findings with clinical variables such as hyposalivation, dental and periodontal status, and the occurrence of oral candidiasis. Methods Participants diagnosed with SjD (SjD group) and age- and sex-matched controls without SjD were included. Sociodemographic data, medication use, unstimulated whole salivary flow (UWSF), plaque index (PI), periodontal screening and recording (PSR), decayed, missing, filled teeth index (DMFT) and use of dental prostheses were assessed. Oral Candida spp. carriage was evaluated through colony-forming unit counts and chromogenic agar cultures from oral rinse samples. Oral microbiota was characterized using 16S rRNA gene amplification and sequencing. Results Clinical data and samples of 61 patients were analysed (SjD group = 27 and control group = 34). Oral health parameters were worse in the SjD group compared with controls. Analysis of microbial diversity in saliva revealed significant differences between the groups. The genera Stenotrophomonas, Streptococcus and Veillonella were more abundant in the SjD group, whereas Haemophilus, Neisseria and Prevotella were more abundant in the control group. The SjD group also exhibited significantly lower overall bacterial diversity, which was associated with greater frequency of moderate and high PI scores and Candida spp. isolates, without association with UWSF. Conclusion These findings of this study suggest that SjD participants presented a dysbiotic and less diverse oral microbiota, and that the observed microbial alterations cannot be explained exclusively by salivary gland-mediated changes in saliva production.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"32 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149049134","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":"Management strategies for articular flares during biologic therapy in non-systemic polyarticular JIA: a multicentre study","authors":"Jonatan Zalcman,Yoel Levinsky,Dana Kenan,Adi Efron,Haran Shani-Narkiss,Irit Tirosh,Amit Ziv,Ruby Haviv,Yonatan Butbul Aviel,Ruth Livny,Gil Amarilyo","doi":"10.1093/rheumatology/keag539","DOIUrl":"https://doi.org/10.1093/rheumatology/keag539","url":null,"abstract":"Abstract Objectives Biologic disease-modifying antirheumatic drugs (bDMARDs) are the cornerstone of treatment for juvenile idiopathic arthritis (JIA), yet the optimal management of flares during ongoing biologic therapy remains unclear. We aimed to compare flare-management strategies and identify risk factors for treatment failure in non-systemic polyarticular JIA. Methods We conducted a multicentre retrospective cohort study across four tertiary paediatric centres in Israel (01/2008–12/2024). We included children with non-systemic polyarticular JIA phenotypes who achieved clinically inactive disease (CID) on a bDMARD and subsequently experienced an articular flare. Treatment success (TS) was defined as achieving CID within 3 months following intervention and maintaining it for ≥3 months. Multivariable logistic regression and Cox proportional hazards models were used to identify predictors of success and time to remission. Results Among 65 patients and 116 flares, five strategies were compared: NSAIDs alone (2.6%), intra-articular corticosteroid injection (IACI; 28.4%), biologic dose optimization (43.1%), IACI plus dose optimization (13.8%), and switching biologics (12.1%). Overall, 71.6% of flares achieved TS. Success rates were highest for IACI (81.8%) and dose optimization (76.0%), and lowest for biologic switching (35.7%). Multivariable analysis identified biologic switching as an independent predictor of lower CID achievement (adjusted OR 0.27; p = 0.036) and significantly delayed remission (HR 0.40; p = 0.006). Conclusions In non-systemic polyarticular JIA, local and incremental strategies like IACI and dose optimization result in higher and faster remission rates compared to immediate biologic switching. A stepwise algorithm prioritizing treatment optimization may prevent unnecessary cycling of biologics.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"106 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149049132","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":"Ultrasensitive quantification links higher serum interferon-α and interferon-γ levels with lower leukocyte and lymphocyte counts in lupus","authors":"Marta Rodríguez-García,Fuensanta Gómez-Bernal,Juan C Quevedo-Abeledo,Beatriz Tejera-Segura,Enrique García-Barrera,Luisa M Villar,Javier Gonzalo Ocejo-Vinyals,Raquel Largo,Miguel Á González-Gay,Iván Ferraz-Amaro","doi":"10.1093/rheumatology/keag529","DOIUrl":"https://doi.org/10.1093/rheumatology/keag529","url":null,"abstract":"Abstract Objectives Hematological abnormalities are common in systemic lupus erythematosus (SLE). Interferon-α (IFN-α) and interferon-γ (IFN-γ) play pivotal roles in the immunopathogenesis of SLE. However, their measurement in serum has long been limited by their extremely low circulating concentrations. With the advent of ultrasensitive detection technologies, these cytokines can now be reliably quantified. In this study, we investigated the association between serum levels of IFN-α and IFN-γ, measured using a highly sensitive assay, and the hematological abnormalities frequently observed in SLE. Methods In this cross-sectional study we recruited 313 patients with SLE. Complete blood counts, including red blood cells, white blood cells, and platelets, were obtained, and serum IFN-α and IFN-γ levels were quantified using the ultrasensitive Single Molecule Array (Simoa) platform. Multivariable linear and logistic regression analyses were performed to evaluate the associations between IFN concentrations and hematological parameters. Results In multivariable analyses, higher serum levels of both IFN-α and IFN-γ were significantly associated with lower total leukocyte, neutrophil, and lymphocyte counts. Platelet and red cells count were not significantly associated with both IFNs. Logistic regression analyses further confirmed that elevated serum concentrations of IFN-α and IFN-γ were independently associated with increased odds of leukopenia and lymphopenia, analyzed as binary outcomes, after adjustment for relevant covariates. Conclusion Elevated serum IFN-α and IFN-γ levels are independently associated with reduced leukocyte and lymphocyte counts in patients with SLE.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"17 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149049133","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":"Unmet needs during the reproductive journey for women with systemic lupus erythematosus.","authors":"Guilherme Ramires de Jesús, Karen Schreiber, Laura Andreoli","doi":"10.1093/rheumatology/keag537","DOIUrl":"https://doi.org/10.1093/rheumatology/keag537","url":null,"abstract":"<p><p>In recent decades, pregnancy in patients with systemic lupus erythematosus (SLE) has ceased to be a contraindication and has become more common due to improved understanding of the disease and improved healthcare assistance. However, there are still many gaps in the reproductive health of women with lupus, ranging from limited guidance on contraception and the best time to conceive, to conflicting information about medication use during pregnancy and concerns regarding short- and long-term complications for both the woman and the child. Finally, access to care for women with lupus remains fragmented, and clinical studies in pregnant women remain limited, which restricts evidence-based medicine in this context. This article reviews the unmet needs during the reproductive journey of women with SLE, discussing ways in which healthcare professionals and administrators can improve care for this group of patients.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149040503","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":"Predicting relapse in pediatric IgA vasculitis in the era of machine learning: an explainable hybrid ensemble model","authors":"Eda Nur Dizman,Zeynep Turgut,Sema Yildirim,Feray Kaya,Elif Kucuk,Lutfiye Koru,Zelal Aydin,Hatice Kubra Dursun,Merve Ozen Balci,Ufuk Furkan Ozdemir,Serpil Meric Toprak,Khatıra Guluzade,Muferet Erguven,Kubra Ozturk,Fatih Haslak","doi":"10.1093/rheumatology/keag533","DOIUrl":"https://doi.org/10.1093/rheumatology/keag533","url":null,"abstract":"Abstract Objectives This study aimed to develop an explainable hybrid ensemble machine learning (ML) model to predict relapse in pediatric IgA vasculitis (IgAV) using routine clinical and laboratory data. Methods This retrospective cohort study included 653 children diagnosed with IgAV according to EULAR/PRINTO/PRES criteria. Demographic, clinical, laboratory data, and the Pediatric Vasculitis Activity Score (PVAS) at diagnosis were evaluated. Several tree-based ML algorithms were evaluated and combined using a soft voting strategy. The hybrid ensemble model combining Random Forest, AdaBoost, and Extra Trees was selected as the final model. SHapley Additive exPlanations (SHAP)-based explainability analysis was performed, and a nomogram was developed for individualized risk prediction. Conventional logistic regression analyses were also performed to identify independent predictors of relapse. Results Relapses were observed in 117 (17.9%) patients during follow-up. The hybrid ensemble model achieved the highest area under the receiver operating characteristic curve (AUC-ROC) (0.7504 ± 0.0856) with competitive area under the precision-recall curve (AUC-PR) (0.4540 ± 0.1308) and Brier score (0.1315 ± 0.0130). SHAP analysis identified older age at diagnosis, higher PVAS, female sex and abnormal urinalysis findings as the most influential predictors of relapse. On conventional regression analysis, PVAS was strongly associated with relapse. In addition, female sex, older age at diagnosis, rash involving the face, and abnormal urinalysis findings were independent predictors. Conclusion Disease relapse in pediatric IgAV can be predicted using clinical and laboratory data with an explainable hybrid ensemble ML approach. The predictive model and nomogram may support risk stratification and targeted follow-up in children with IgAV.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"47 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149020571","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":"Tocilizumab for severe gout-induced septic-like syndrome in the intensive care unit: a case report","authors":"Qitian Ou,Yujun Deng,Cheng Sun,Hongke Zeng,Wenhong Zhong","doi":"10.1093/rheumatology/keag534","DOIUrl":"https://doi.org/10.1093/rheumatology/keag534","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"40 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149020570","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":"Outcomes of intensive care patients with acute small-vessel vasculitis: a 23-year single-centre experience","authors":"Arden Dierker Viik,Yiwang Xu,Dominic McGovern,Seerapani Gopaluni,Michael Chen-Xu,James Varley,Lisa Willcocks,Rona Smith,Iftach Sagy,David Jayne,Rachel Jones","doi":"10.1093/rheumatology/keag531","DOIUrl":"https://doi.org/10.1093/rheumatology/keag531","url":null,"abstract":"Abstract Objectives Patients with fulminant small-vessel vasculitis (SVV) may require intensive care unit (ICU) admission, where mortality is high. We describe outcomes and prognostic factors in ICU patients with ANCA-associated vasculitis (AAV) and anti-glomerular basement membrane (anti-GBM) disease over a 23-year period. Methods Patients admitted with acute manifestations of new or flaring AAV or anti-GBM disease were identified from our hospital ICU database (1999-2022). Patients treated solely in high-dependency areas were excluded. Primary outcomes were mortality at 30 and 90 days and development of end-stage kidney disease (ESKD). A matched case-control analysis (1:4) adjusted for age, gender, APACHE II score and admission year was performed for a patient subgroup. Results 85 cases (82% AAV, 15% anti-GBM) were identified, 80% newly diagnosed. Median APACHE II score was 19. Ventilatory support and continuous venovenous haemodiafiltration were required in 92% and 66% of cases, respectively. 30-day and 90-day mortality rates were 15% and 26%, respectively. Among 90-day survivors, 17 (28%) developed new ESKD. Vasculitis treatments included cyclophosphamide (59%), rituximab (53% of AAV patients) and plasma exchange (71%). These treatments were associated with 90-day and long-term survival. Treatment for vasculitis post-2008 was associated with 90-day and long-term survival, which was not mirrored in ICU controls. This coincided with improvements to the vasculitis service and therapeutic strategies. Conclusions This single-centre study reports ICU outcomes for patients with fulminant SVV over 23 years. 30 and 90-day ICU mortality were comparable with other reported values. Improvement in mortality was associated with changes in vasculitis treatment protocols.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"8 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149020607","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}