{"title":"Early mepolizumab initiation during remission induction is associated with lower organ damage burden in EGPA: a multicentre cohort study.","authors":"Akihito Maruyama, Nobuyuki Ono, Tatsuya Kai, Yasutaka Kimoto, Miki Nakano, Yasushi Inoue, Naoyasu Ueda, Tomoya Miyamura, Kyoko Otsuka, Yuya Shinkawa, Yoshifumi Tada, Mitsuteru Akahoshi, Hiroaki Niiro","doi":"10.1093/rheumatology/keag523","DOIUrl":"10.1093/rheumatology/keag523","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this study was to evaluate whether early mepolizumab initiation during remission induction is associated with favourable clinical outcomes and lower organ damage burden in patients with eosinophilic granulomatosis with polyangiitis (EGPA).</p><p><strong>Methods: </strong>This retrospective multicentre cohort study used data from the Kyushu Vasculitis Cohort Study. Patients with EGPA were classified into an early mepolizumab group, in which mepolizumab was initiated within 3 months of remission induction, and a conventional group. The primary end point was clinical remission at 1 year, defined as BVAS = 0 with prednisolone ≤4 mg/day. Secondary end points included remission at 6 months and 2 years, glucocorticoid exposure, relapse, mortality, and organ damage as assessed by the Vasculitis Damage Index (VDI). Longitudinal VDI values from 6 months to 2 years were analysed using a linear mixed-effects model.</p><p><strong>Results: </strong>Sixty-four patients were included (early mepolizumab, n = 16; conventional, n = 48). Clinical remission at 1 year was achieved more frequently in the early mepolizumab group than in the conventional group (69% vs 27%; P = 0.006). At 2 years, remission was achieved in 10/10 (100%) vs 13/45 (29%) evaluable patients (P < 0.001), and glucocorticoid discontinuation was achieved in 4/10 (40%) vs 4/45 (9%) of those patients (P = 0.029). BVAS decreased markedly in both groups. Early mepolizumab initiation was associated with lower longitudinal VDI values (β = -0.36, 95% CI -0.70 to -0.03; P = 0.032).</p><p><strong>Conclusion: </strong>Early mepolizumab initiation was associated with higher remission rates, lower glucocorticoid exposure and lower organ damage burden during the first 2 years in EGPA.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13653373/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012811","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}
RheumatologyPub Date : 2026-10-01DOI: 10.1093/rheumatology/keag509
Jing Jing Wang, Alexander Troelnikov, Bridie Armour, Caizhu Teng, Tim Chataway, Véronique le Guern, Eric Hachulla, Valérie Devauchelle-Pensec, Claire Larroche, Jacques-Eric Gottenberg, Xavier Mariette, Gaetane Nocturne
{"title":"A specific molecular profiling of secreted rheumatoid factors in Sjögren's disease patients with lymphoma.","authors":"Jing Jing Wang, Alexander Troelnikov, Bridie Armour, Caizhu Teng, Tim Chataway, Véronique le Guern, Eric Hachulla, Valérie Devauchelle-Pensec, Claire Larroche, Jacques-Eric Gottenberg, Xavier Mariette, Gaetane Nocturne","doi":"10.1093/rheumatology/keag509","DOIUrl":"10.1093/rheumatology/keag509","url":null,"abstract":"<p><strong>Objective: </strong>To perform proteomic analysis of secreted IgM-rheumatoid factor (RF) in patients with Sjögren's disease (SjD) with and without lymphoma to determine whether a specific RF molecular profile is associated with lymphoma occurrence.</p><p><strong>Methods: </strong>We studied 23 RF+ SjD patients from the ASSESS cohort. Eleven had lymphoma (cases), including five collected prior to lymphoma diagnosis (pre-lymphoma), and 12 had no lymphoma (controls). Cases and controls were matched for age and gender. Serum RF heavy chains were purified and analysed using de novo mass spectrometry and database matching. IGHV subfamilies were identified, and amino acid sequences compared between cases and controls. Full RF sequences were used for structural modelling and affinity prediction.</p><p><strong>Results: </strong>We observed a progressive enrichment of detectable immunoglobulin heavy chain variable region (VH) subfamilies among circulating RFs, with over-representation of VH1-69 RFs in SjD patients with lymphoma compared with those without lymphoma. We observed fewer somatic mutations in VH1-69 RFs from pre lymphoma SjD patients whilst IGHV3 family RFs contained a greater number of unique mutations. Focused on VH169s, we identified convergence of mutations amongst all patients and identified less frequent antigen-interacting mutations in pre-lymphoma patients.</p><p><strong>Conclusion: </strong>This study suggests changes in the serum RF antibody repertoire in SjD patients prior to and following lymphoma diagnosis, offering new insights into the serum clonal dynamics of the disease. Compositional changes in circulating RF clones may warrant further investigation in larger, independent cohorts to assess their potential value as a predictive biomarker for lymphoma.</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":"148983673","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}
RheumatologyPub Date : 2026-10-01DOI: 10.1093/rheumatology/keag519
Wei Huang, Xing-Yu Wan, Yi-Xu Wang, Yi-Han Hu, Shi-Hao Jing, Yi Zhang, Pan Ke, Dan-Kang Li, Chao Zhang, Rui-Xue Leng, Qi Wang, Dong-Sheng Di
{"title":"Early-life tobacco smoke exposure and incident rheumatoid arthritis: mediated by allostatic load and joint effects with polygenic risk.","authors":"Wei Huang, Xing-Yu Wan, Yi-Xu Wang, Yi-Han Hu, Shi-Hao Jing, Yi Zhang, Pan Ke, Dan-Kang Li, Chao Zhang, Rui-Xue Leng, Qi Wang, Dong-Sheng Di","doi":"10.1093/rheumatology/keag519","DOIUrl":"10.1093/rheumatology/keag519","url":null,"abstract":"<p><strong>Objectives: </strong>RA is a widespread autoimmune disorder. Early-life environmental exposures may contribute to RA, and tobacco smoke represents an important modifiable factor. This study aimed to examine whether early-life tobacco smoke exposure affects incident RA risk, and to clarify the potential mediating role of allostatic load (AL) and interactive effects with genetic susceptibility.</p><p><strong>Methods: </strong>Using questionnaire, biomarker, genetic, and linked health-record data from UK Biobank, we examined intrauterine tobacco smoke exposure (IUTSE; n = 427 037), age at tobacco use initiation (ATUI; n = 414 318) and RA cases. Cox models were used to evaluate associations between early-life tobacco exposure and RA risk. Mediation analysis quantified the mediating effect of AL. Additive and multiplicative interaction examined genetic susceptibility interactions.</p><p><strong>Results: </strong>IUTSE was associated with higher RA risk [hazard ratio (HR) = 1.15, 95% CI: 1.08, 1.21]. Compared with never-smokers, individuals who started tobacco smoking in childhood, adolescence, and adulthood exhibited elevated RA risk, with corresponding HRs of 1.74 (95% CI: 1.39, 2.17), 1.56 (95% CI: 1.29, 1.88) and 1.47 (95% CI: 1.22, 1.76), respectively. Mediation analyses indicated statistically significant but small indirect effects of AL. The indirect effect for IUTSE was 1.001 (95% CI: 1.001, 1.002) and the indirect effects for tobacco use during childhood, adolescence and adulthood were 1.009 (95% CI: 1.005, 1.013), 1.006 (95% CI: 1.003, 1.009) and 1.006 (95% CI: 1.003, 1.009), respectively. High genetic risk with IUTSE (HR = 1.93, 95% CI: 1.75, 2.13) or childhood initiation (HR = 2.89, 95% CI: 2.17, 3.86) increased RA risk.</p><p><strong>Conclusion: </strong>Early-life tobacco smoke exposure elevates RA risk in adulthood, with limited mediation by AL and modification by genetic predisposition.</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":"149002502","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}
RheumatologyPub Date : 2026-10-01DOI: 10.1093/rheumatology/keag576
Sabrina Hundal, Julian Cappelli, Mohammed Kaouache, Jessica Muha, Savanah Haidar, Lorenzo Beretta, Zahi Touma, John S Barbieri, Stephanie R Goldberg, Anastasiya Muntyanu, Ioannis Parodis, Elena Netchiporouk
{"title":"Responsiveness of patient-reported outcomes by disease trajectory in SLE.","authors":"Sabrina Hundal, Julian Cappelli, Mohammed Kaouache, Jessica Muha, Savanah Haidar, Lorenzo Beretta, Zahi Touma, John S Barbieri, Stephanie R Goldberg, Anastasiya Muntyanu, Ioannis Parodis, Elena Netchiporouk","doi":"10.1093/rheumatology/keag576","DOIUrl":"10.1093/rheumatology/keag576","url":null,"abstract":"<p><strong>Objectives: </strong>SLE is a heterogeneous autoimmune disease with persistent symptoms that profoundly impair quality of life yet are often under-recognized by clinician-assessed instruments. Patient-reported outcomes (PROs) are increasingly incorporated in clinical trials to capture treatment benefit from the patient perspective. However, the responsiveness of PRO instruments and their alignment with clinician-assessed disease activity across distinct SLE trajectories remain incompletely defined. We evaluated the longitudinal responsiveness and convergent validity of PRO measures across predefined disease trajectory classes.</p><p><strong>Methods: </strong>Secondary analysis of pooled patient-level data from two phase III randomized clinical trials of belimumab in adults with active SLE (n = 1908), stratified into four previously derived trajectory classes defined by baseline disease activity and treatment response. PROs included Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue), 36-Item Short Form Survey (SF-36) Physical Component Summary (PCS) and Mental Component Summary (MCS), EuroQol 5-Dimension 3-level (EQ-5D-3L) and EuroQol Visual Analogue Scale (EQ-VAS). Responsiveness was quantified using standardized response means (SRMs). Convergent validity was evaluated using repeated-measures correlations between within-person changes in PROs and clinician-assessed disease activity measures (SLEDAI-2K, BILAG and Physician's Global Assessment [PGA]), with sensitivity analyses performed to assess the robustness of findings.</p><p><strong>Results: </strong>FACIT-F and SF-36 PCS generally demonstrated the greatest responsiveness, while EQ-5D-3L showed smaller changes and less consistent responsiveness across classes and timepoints. FACIT-F demonstrated early responsiveness, whereas SF-36 PCS showed sustained responsiveness over later follow-up. PRO changes were most consistently associated with PGA. Sensitivity analyses showed broadly similar patterns.</p><p><strong>Conclusion: </strong>FACIT-F and SF-36 PCS demonstrated the greatest responsiveness and alignment with clinician-assessed disease activity. EQ-5D-3L showed less consistent responsiveness, suggesting domain-specific PROs may better capture treatment benefit in SLE trials and health technology assessments.</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":"149196312","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}
RheumatologyPub Date : 2026-10-01DOI: 10.1093/rheumatology/keag582
Oh Chan Kwon, Hye Sun Lee, So Young Jeon, Min-Chan Park
{"title":"Cardiovascular risk associated with first-line Janus kinase inhibitors versus TNF inhibitors in patients with RA: a nationwide cohort study.","authors":"Oh Chan Kwon, Hye Sun Lee, So Young Jeon, Min-Chan Park","doi":"10.1093/rheumatology/keag582","DOIUrl":"10.1093/rheumatology/keag582","url":null,"abstract":"<p><strong>Objective: </strong>To compare the risk of major adverse cardiovascular events (MACEs) between TNF inhibitors (TNFi) and Janus kinase inhibitors (JAKi) when initiated as first-line advanced therapy in patients with RA.</p><p><strong>Methods: </strong>Using the Korean nationwide claims database, we identified patients newly diagnosed with RA between 2010 and 2015. Patients who initiated TNFi or JAKi as first-line advanced therapy were selected for analysis. Patients were followed from initiation of TNFi or JAKi through June 2024. The primary outcome was incident MACE, defined as myocardial infarction, ischaemic stroke or coronary revascularization. Multivariable Cox proportional hazards models were applied in the total cohort. We additionally performed propensity score (PS)-matched analyses to address confounding by indication. Prespecified subgroup analyses were conducted according to baseline cardiovascular risk factors.</p><p><strong>Results: </strong>The study included 6174 patients (TNFi = 5152; JAKi = 1022). The incidence rates of MACE were 4.1 and 3.6 per 1000 person-years in the TNFi and JAKi groups, respectively. MACE risk did not differ significantly between JAKi and TNFi in the total study cohort (adjusted hazard ratio [HR] 0.636, 95% CI 0.283-1.429) or in the PS-matched population (adjusted HR 0.715, 95% CI 0.187-2.739). Results were consistent across all prespecified subgroups, including patients with baseline cardiovascular risk factors.</p><p><strong>Conclusion: </strong>First-line use of JAKi was not associated with an increased risk of MACE compared with TNFi. These findings provide real-world evidence supporting the cardiovascular safety of JAKi as first-line advanced therapy in patients with RA.</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":"149207571","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}
RheumatologyPub Date : 2026-10-01DOI: 10.1093/rheumatology/keag521
Francesco Bonomi, Giulia Bandini, Corrado Campochiaro, Veronica De Silvestri, Svetlana Agachi, Christina Bergmann, Oliver Distler, Brigitte Granel, Joerg Henes, Florenzo Iannone, Luca Idolazzi, Eduardo Kerzberg, Mohammad E Naffaa, Hadi Poormoghim, Mislav Radic, Marie-Elise Truchetet, Silvia Bellando Randone, Marco Matucci Cerinic, Zsuzsanna H McMahan, Michael Hughes
{"title":"Proton pump inhibitor use and long-term outcomes in SSc: a real-life analysis from the EUSTAR database.","authors":"Francesco Bonomi, Giulia Bandini, Corrado Campochiaro, Veronica De Silvestri, Svetlana Agachi, Christina Bergmann, Oliver Distler, Brigitte Granel, Joerg Henes, Florenzo Iannone, Luca Idolazzi, Eduardo Kerzberg, Mohammad E Naffaa, Hadi Poormoghim, Mislav Radic, Marie-Elise Truchetet, Silvia Bellando Randone, Marco Matucci Cerinic, Zsuzsanna H McMahan, Michael Hughes","doi":"10.1093/rheumatology/keag521","DOIUrl":"10.1093/rheumatology/keag521","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the association between proton pump inhibitor (PPI) use, all-cause mortality and interstitial lung disease (ILD) progression in SSc.</p><p><strong>Methods: </strong>SSc patients enrolled in the EUSTAR registry with at least two clinical visits were included (EUSTAR CP141). PPI exposure was modelled as a time-varying variable. Inverse probability of treatment weighting (IPTW) based on propensity scores was applied to address confounding by indication. All-cause mortality was analysed using IPTW-weighted time-varying Cox models. ILD progression was assessed using functional endpoints (FVC decline ≥10% and ≥5%; DLCO decline ≥10%). Restricted mean survival time (RMST) was used to quantify absolute effects. Sensitivity analyses included a 6-month lag time.</p><p><strong>Results: </strong>Out of 10 660 patients enrolled, 8637 (81.0%) were exposed to PPIs during a mean follow-up of 5.7 ± 4.3 years. They exhibited a more severe multisystem disease phenotype, including greater gastrointestinal, pulmonary and vascular involvement. After adjustment, PPI exposure was associated with higher all-cause mortality (HR 1.88, 95% CI 1.40-2.54), although the RMST difference at 5 years was minimal. As cause-specific mortality was unavailable, this association might reflect underlying disease severity rather than a direct drug effect. The use of PPI was not associated with a reduced risk of clinically meaningful FVC decline, while a modest attenuation of DLCO decline was observed, and absolute progression-free differences were limited.</p><p><strong>Conclusions: </strong>PPI is commonly used in SSc characterized by a more severe multisystem phenotype. Only a marginal attenuation of ILD progression is observed, although absolute effects were small. These findings favour the generalized use of PPIs for symptomatic gastroesophageal reflux in SSc.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13637600/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149002653","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}
RheumatologyPub Date : 2026-10-01DOI: 10.1093/rheumatology/keag551
Hamzah Amin,Marwan Bukhari
{"title":"Is Trabecular bone score associated with multiple fragility fractures? Evidence from a large observational cohort study","authors":"Hamzah Amin,Marwan Bukhari","doi":"10.1093/rheumatology/keag551","DOIUrl":"https://doi.org/10.1093/rheumatology/keag551","url":null,"abstract":"Abstract Objectives Trabecular bone score (TBS) is a measure of bone microarchitecture associated with hip and major osteoporotic fractures. However, TBS has not been evaluated in UK-specific cohorts, and little is known about its association with multiple fragility fracture phenotypes; this study aimed to investigate these associations. Methods TBS was available in a subset of patients referred for DXA assessment in northwest England between June 2004 and May 2025. Fragility fracture phenotypes were defined as a single hip fracture (SHF), single non-hip fracture (SNHF), multiple non-hip fractures (MNHF), and multiple fractures including at least one hip fracture (MFH). These categories served as the dependent variable in a multinomial logistic regression model. Ordinal logistic regression models assessed associations with ordered hip and non-hip fracture categories. All models were adjusted for FRAX variables and the mean TBS, with multiple comparisons accounted for using Bonferroni correction. Results A total of 2,782 patients were included, with a mean age of 66.84 years (SD 12.31), mean BMI of 27.31 kg/m² (SD 5.91), and 2,146 (77.2%) were female. Lower TBS was associated with SNHF (OR 1.37, 95% CI 1.28–1.48), MNHF (OR 1.32, 95% CI 1.27–1.36), and MFH (OR 1.68, 95% CI 1.16–2.43). In ordinal models, lower TBS was associated with increasing fracture burden within the non-hip fracture groups (OR 1.31, 95% CI 1.16–1.47), but was not associated with SHF or the hip fracture groups. Conclusion TBS is associated with multiple fragility fracture phenotypes and greater fracture burden within non-hip fracture categories.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"53 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149089278","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}
RheumatologyPub Date : 2026-10-01DOI: 10.1093/rheumatology/keag516
Nicola Farina, Silvia Bellando Randone, Hilde Jenssen-Bjørkekjær, Sebastien Sanges, Patricia Carreira, Paolo Airò, Serena Guiducci, Dilia Giuggioli, Gabriela Riemekasten, Carmen-Pilar Simeón Aznar, Christina Bergmann, Elise Siegert, Ivan Castellví, Lesley Ann Saketkoo, Jeska de Vries-Bouwstra, Philipp Klemm, Ulf Müller-Ladner, Alexandra Balbir-Gurman, Vanessa Smith, Florenzo Iannone, Luca Idolazzi, Christopher Denton, Edoardo Rosato, Britta Maurer, Yannick Allanore, Yoshiya Tanaka, Elisabetta Zanatta, Marie-Elise Truchetet, Masataka Kuwana, Mickaël Martin, Alberto Cauli, Kamal Solanki, Francesco Del Galdo, Ana Maria Gheorghiu, Branimir Anic, Gonçalo Boleto, Gábor Kumánovics, Kristofer Andréasson, Simona Rednic, Lorinda S Chung, Susana Oliveira, Marius Cadar, Addolorata Corrado, Carolina de Souza Müller, Vivien M Hsu, Yair Levy, Gianluca Moroncini, Jörg Henes, Andra Balanescu, Ellen De Langhe, Carlomaurizio Montecucco, Petros P Sfikakis, Michele Iudici, Stefan Heitmann, Madelon C Vonk, Anna-Maria Hoffmann-Vold, Oliver Distler, Marco Matucci Cerinic, Cosimo Bruni
{"title":"Effectiveness of oral anticoagulants in precapillary pulmonary hypertension associated with systemic sclerosis: a EUSTAR cohort study.","authors":"Nicola Farina, Silvia Bellando Randone, Hilde Jenssen-Bjørkekjær, Sebastien Sanges, Patricia Carreira, Paolo Airò, Serena Guiducci, Dilia Giuggioli, Gabriela Riemekasten, Carmen-Pilar Simeón Aznar, Christina Bergmann, Elise Siegert, Ivan Castellví, Lesley Ann Saketkoo, Jeska de Vries-Bouwstra, Philipp Klemm, Ulf Müller-Ladner, Alexandra Balbir-Gurman, Vanessa Smith, Florenzo Iannone, Luca Idolazzi, Christopher Denton, Edoardo Rosato, Britta Maurer, Yannick Allanore, Yoshiya Tanaka, Elisabetta Zanatta, Marie-Elise Truchetet, Masataka Kuwana, Mickaël Martin, Alberto Cauli, Kamal Solanki, Francesco Del Galdo, Ana Maria Gheorghiu, Branimir Anic, Gonçalo Boleto, Gábor Kumánovics, Kristofer Andréasson, Simona Rednic, Lorinda S Chung, Susana Oliveira, Marius Cadar, Addolorata Corrado, Carolina de Souza Müller, Vivien M Hsu, Yair Levy, Gianluca Moroncini, Jörg Henes, Andra Balanescu, Ellen De Langhe, Carlomaurizio Montecucco, Petros P Sfikakis, Michele Iudici, Stefan Heitmann, Madelon C Vonk, Anna-Maria Hoffmann-Vold, Oliver Distler, Marco Matucci Cerinic, Cosimo Bruni","doi":"10.1093/rheumatology/keag516","DOIUrl":"10.1093/rheumatology/keag516","url":null,"abstract":"<p><strong>Objectives: </strong>The role of anticoagulation in systemic sclerosis (SSc) with precapillary pulmonary hypertension (PH) remains controversial, with conflicting evidence regarding its impact on survival, including potential harmful effects. Aiming to address this research gap, we evaluated both mortality and PH worsening in a large SSc-precapillary PH cohort, additionally accounting for SSc-specific risk factors in our analyses, to better inform individualized treatment decisions.</p><p><strong>Methods: </strong>This retrospective cohort study included SSc patients from the European Scleroderma Trials and Research database with precapillary PH confirmed by right heart catheterization (RHC). The association of oral anticoagulants (OAC) use with survival and PH worsening was assessed using Kaplan-Meier estimates and multivariable Cox regression models. To minimize confounding, propensity score matching was additionally performed.</p><p><strong>Results: </strong>Among 614 patients included, 143 (23%) received OAC at the time of RHC. At baseline, patients on OAC had worse hemodynamic parameters. No significant association was found between OAC use and survival (HR 0.983 95%CI 0.724-1.334) or PH worsening (HR 1.070 95%CI 0.811-1.412). These findings remained consistent after propensity score matching and in the subgroup of 230 SSc patients with precapillary PH without interstitial lung disease.</p><p><strong>Conclusion: </strong>In this large SSc cohort with precapillary PH, we did not observe any clinical benefit associated with OAC, neither in terms of survival nor of PH worsening. Further prospective studies are needed to determine whether specific patient subgroups may benefit from anticoagulation therapy.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13657258/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148991206","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}