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Characteristics and outcomes of anti-Ku antibody-associated interstitial lung disease: a retrospective study of 60 patients. 抗ku抗体相关间质性肺疾病的特点和预后:60例患者的回顾性研究
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-27 DOI: 10.1136/rmdopen-2026-006943
Marie Robert, Yann Nguyen, Marie-Pierre Debray, Raphael Borie, Yurdagül Uzunhan, Yves Allenbach, Karim Sacré, Benjamin Terrier, Zahir Amoura, Céline Comparon, Philippe Dieudé, Véronique Le Guern, Capucine Morélot-Panzini, Marc Humbert, Olivier Sitbon, Cécile Goujard, Brigitte Bader-Meunier, Bruno Fautrel, Jérome Hadjadj, Pascale Chrétien, Pascale Roland-Nicaise, Claire Goulvestre, Jean-Luc Charuel, Olivier Benveniste, Luc Mouthon, Victoire De Lastours, Perrine Dusser, Mohamad Zaidan, Elisabeth Aslangul, Marie Saillour, Glory Dingulu, François Chasset, Thach Bui-Quang, Jacques Cadranel, Gaétane Nocturne, Xavier Mariette, Samuel Bitoun, Pierre-Yves Brillet, Raphaèle Seror
{"title":"Characteristics and outcomes of anti-Ku antibody-associated interstitial lung disease: a retrospective study of 60 patients.","authors":"Marie Robert, Yann Nguyen, Marie-Pierre Debray, Raphael Borie, Yurdagül Uzunhan, Yves Allenbach, Karim Sacré, Benjamin Terrier, Zahir Amoura, Céline Comparon, Philippe Dieudé, Véronique Le Guern, Capucine Morélot-Panzini, Marc Humbert, Olivier Sitbon, Cécile Goujard, Brigitte Bader-Meunier, Bruno Fautrel, Jérome Hadjadj, Pascale Chrétien, Pascale Roland-Nicaise, Claire Goulvestre, Jean-Luc Charuel, Olivier Benveniste, Luc Mouthon, Victoire De Lastours, Perrine Dusser, Mohamad Zaidan, Elisabeth Aslangul, Marie Saillour, Glory Dingulu, François Chasset, Thach Bui-Quang, Jacques Cadranel, Gaétane Nocturne, Xavier Mariette, Samuel Bitoun, Pierre-Yves Brillet, Raphaèle Seror","doi":"10.1136/rmdopen-2026-006943","DOIUrl":"10.1136/rmdopen-2026-006943","url":null,"abstract":"<p><strong>Objective: </strong>Anti-Ku antibodies (Abs) are rare and detected in various autoimmune diseases (AIDs), presenting in various phenotypes, potentially affecting different organs including the lungs. This study aimed to describe the characteristics and evolution of interstitial lung disease (ILD) in anti-Ku-positive patients.</p><p><strong>Methods: </strong>An observational, multicentre, retrospective study was conducted across 10 French University Hospitals between January 2010 and June 2025, including patients with anti-Ku Abs with ILD. Clinical data and all pulmonary function tests and chest CT scans available were reviewed for this study. ILD progression was defined using criteria inspired by the American Thoracic Society/European Respiratory Society/Japenese Respiratory Society/Asociación Latinoamericana de Tórax Clinical Practice Guidelines, applied across the entire follow-up period rather than within the 12-month timeframe, to capture all clinically meaningful progression events. To account for event timing, time to ILD progression was analysed as a time-to-event outcome using Cox proportional hazards models.</p><p><strong>Results: </strong>Among 154 anti-Ku-positive patients (51 with idiopathic inflammatory myopathy, 46 with systemic lupus erythematosus, 30 with Sjögren's disease, 27 with systemic sclerosis), 60 (39%) had ILD (68% women, median age 56 years). The predominant ILD pattern was fibrotic non-specific interstitial pneumonia (27%, n=16). ILD was already present at AID diagnosis in 48 patients (80%). Forty-five (75%) patients progressed after a median time of 4 (2-11) years. Male sex (adjusted HR (aHR) 2.7; 95% CI 1.4 to 5.2) was associated with ILD progression. When the 12-month cut-off was strictly applied, only six patients fulfilled the progressive fibrosing ILD definition. Baseline pulmonary fibrosis was present in 34 (57%) patients and was associated with a reduced survival when adjusting for age at ILD diagnosis and cardiac involvement (aHR 6.5, 95% CI 1.5 to 28.2).</p><p><strong>Conclusion: </strong>ILD occurred in 39% of anti-Ku-positive patients and progressed in 75% of them, underscoring the importance of systematic ILD screening and monitoring in these patients.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13409090/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607158","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}
引用次数: 0
Oral microbiome dysbiosis and oral-gut microbial network disruption in hand osteoarthritis: data from the Xiangya Osteoarthritis Study. 手骨关节炎的口腔微生物群失调和口腔肠道微生物网络破坏:来自湘雅骨关节炎研究的数据。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-21 DOI: 10.1136/rmdopen-2026-006962
Jiatian Li, Yadan Xiao, Tuo Yang, David J Hunter, Weiya Zhang, Michael Doherty, Yuqing Zhang, Zidan Yang, Yilun Wang, Dongxing Xie, Changjun Li, Kai Zhao, Wei Li, Zeqin Wen, Chao Zeng, Guanghua Lei, Jie Wei
{"title":"Oral microbiome dysbiosis and oral-gut microbial network disruption in hand osteoarthritis: data from the Xiangya Osteoarthritis Study.","authors":"Jiatian Li, Yadan Xiao, Tuo Yang, David J Hunter, Weiya Zhang, Michael Doherty, Yuqing Zhang, Zidan Yang, Yilun Wang, Dongxing Xie, Changjun Li, Kai Zhao, Wei Li, Zeqin Wen, Chao Zeng, Guanghua Lei, Jie Wei","doi":"10.1136/rmdopen-2026-006962","DOIUrl":"10.1136/rmdopen-2026-006962","url":null,"abstract":"<p><strong>Objectives: </strong>The oral microbiome plays a critical role in modulating systemic inflammation, partly through its interactions with the gut microbiome. Although gut microbiome dysbiosis has been implicated in symptomatic hand osteoarthritis (SHOA), the role of oral microbiome dysbiosis in SHOA and its relationship with gut microbiome dysbiosis remain unclear. Elucidating these associations could provide novel insights into SHOA pathogenesis.</p><p><strong>Methods: </strong>Participants were recruited from the Xiangya Osteoarthritis (XO) Study, an ongoing community-based observational study. Saliva samples were analysed using 16S ribosomal RNA gene sequencing. Oral microbial richness, composition and relative abundance of specific taxa were compared between SHOA participants and controls without SHOA. Correlations within the oral-gut microbiome network were also assessed and compared between groups.</p><p><strong>Results: </strong>Compared with controls (n=712), participants with SHOA (n=52) exhibited significantly lower oral microbial richness (p=0.007) and altered composition (p=0.007). The relative abundance of the genus <i>Trichococcus</i> was significantly higher in SHOA participants (β=0.437 (95% CI 0.174 to 0.699), p=0.001, Q=0.073) and positively associated with SHOA severity. Furthermore, the number of significant correlations within the oral-gut microbiome network was markedly reduced in SHOA participants compared with controls. Notably, <i>Trichococcus</i> abundance in the oral microbiome correlated positively with the gut microbial KEGG pathway of tyrosine metabolism (r=0.137, p=0.001, Q=0.047), both linked to SHOA.</p><p><strong>Conclusion: </strong>Oral microbiome dysbiosis and disruption of the oral-gut microbiome network are associated with prevalent SHOA. These findings suggest a potential role of the oral-gut microbiome axis in SHOA pathogenesis. Larger studies are needed to confirm these associations.</p><p><strong>Trial registration number: </strong>NCT04033757.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13410909/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148550295","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}
引用次数: 0
Refining serological scores in rheumatoid arthritis classification: an international study. 类风湿关节炎分类的精细化血清学评分:一项国际研究。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-20 DOI: 10.1136/rmdopen-2026-007007
Lieve Van Hoovels, Christos Chatzichristos, Juan Irure-Ventura, Daphné Vandebeek, Elke Lodewijckx, Bert Vander Cruyssen, Daniela Sieghart, Carolien Bonroy, Eszter Nagy, Rille Pullerits, Saša Čučnik, Charlotte Dahle, Ingmar Heijnen, Luca Bernasconi, Farid Benkhadra, Laura Bogaert, Stefanie Van Den Bremt, Ann Van Liedekerke, Geert Vanheule, Johan Robbrecht, Claudine Wirth, Rüdiger B Mueller, Diego Kyburz, Christopher Sjöwall, Alf Kastbom, Rok Ješe, Boja Jovancevic, Emese Kiss, Peggy Jacques, Maarten De Vos, Marcos López-Hoyos, Günter Steiner, Patrick Verschueren, Xavier Bossuyt
{"title":"Refining serological scores in rheumatoid arthritis classification: an international study.","authors":"Lieve Van Hoovels, Christos Chatzichristos, Juan Irure-Ventura, Daphné Vandebeek, Elke Lodewijckx, Bert Vander Cruyssen, Daniela Sieghart, Carolien Bonroy, Eszter Nagy, Rille Pullerits, Saša Čučnik, Charlotte Dahle, Ingmar Heijnen, Luca Bernasconi, Farid Benkhadra, Laura Bogaert, Stefanie Van Den Bremt, Ann Van Liedekerke, Geert Vanheule, Johan Robbrecht, Claudine Wirth, Rüdiger B Mueller, Diego Kyburz, Christopher Sjöwall, Alf Kastbom, Rok Ješe, Boja Jovancevic, Emese Kiss, Peggy Jacques, Maarten De Vos, Marcos López-Hoyos, Günter Steiner, Patrick Verschueren, Xavier Bossuyt","doi":"10.1136/rmdopen-2026-007007","DOIUrl":"10.1136/rmdopen-2026-007007","url":null,"abstract":"<p><strong>Background: </strong>In the American College of Rheumatology/European Alliance of Rheumatology Associations 2010 classification criteria for rheumatoid arthritis (RA), rheumatoid factor (RF) and anti-citrullinated protein/peptide antibodies (ACPA) are assigned the same weight, although ACPA have proven more specific than RF. We aimed to refine the serological weight factors for RA classification.</p><p><strong>Materials and methods: </strong>Diagnostic samples from 398 patients with RA and 448 diseased controls (DC) were evaluated with RF and ACPA assays from five different manufacturers. A grid search was conducted across all possible integer score pairs for RF and ACPA to identify the weighting combination that optimised the specificity of RA classification. This combination was applied in a refined scoring system (RSS) for three confirmatory cohorts comprising, respectively, 67, 157 and 132 patients with RA and 441, 153 and 193 DC.</p><p><strong>Findings: </strong>In the investigative cohort, the optimal combination of weighting scores was characterised by higher scores for ACPA, higher antibody levels and double RF/ACPA positivity.Applying the optimal combination of weighting scores decreased misclassification compared with the 2010 RA classification criteria, minimally affecting sensitivity. For seropositive patients, RSS significantly lowered misclassification from 38.3-55.8% to 15.0-26.9%.The improved specificity of RSS (compared with the 2010 criteria) was validated in the confirmatory cohorts. Considering only seropositive patients, the RSS significantly decreased RA misclassification (8.6-36.8% vs 20.0-78.9%), minimally affecting the sensitivity (86.7-97.3% vs 86.7-96.0%).</p><p><strong>Interpretation: </strong>Serological weight factors for RA classification can be improved by taking into account the antibody type, the antibody levels and single or combined positivity. The application of an RSS reduced the number of RA misclassifications.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13386081/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148536758","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}
引用次数: 0
Medication adherence in Behçet's syndrome: real-world data from a tertiary referral cohort. behet综合征的药物依从性:来自三级转诊队列的真实数据。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-20 DOI: 10.1136/rmdopen-2026-006982
Rosaria Talarico, Federica Di Cianni, Antonello Sulis, Giulia Lubrano Lavadera, Diana Marinello, Marta Mosca
{"title":"Medication adherence in Behçet's syndrome: real-world data from a tertiary referral cohort.","authors":"Rosaria Talarico, Federica Di Cianni, Antonello Sulis, Giulia Lubrano Lavadera, Diana Marinello, Marta Mosca","doi":"10.1136/rmdopen-2026-006982","DOIUrl":"10.1136/rmdopen-2026-006982","url":null,"abstract":"<p><strong>Objectives: </strong>Medication adherence is a key determinant of treatment effectiveness in chronic inflammatory diseases but data in Behçet's syndrome (BS) remain limited. This study evaluated medication adherence in a real-world cohort of patients with BS and explored demographic and clinical factors associated with reduced adherence.</p><p><strong>Methods: </strong>We conducted a monocentric cross-sectional study including 125 patients with BS followed at a tertiary referral centre. Adherence was assessed using the validated 8-item Morisky Medication Adherence Scale (MMAS-8). Patients were classified as having high (score=8), intermediate (score 6-<8) or low adherence (score<6). Disease activity was evaluated using the Behçet's Disease Current Activity Form and the Behçet's Disease Activity Index. Associations between adherence and clinical variables were analysed using Spearman correlation and non-parametric tests.</p><p><strong>Results: </strong>The mean MMAS-8 Score was 6.82±1.32. High adherence was observed in 29.6% of patients, intermediate adherence in 48.8% and low adherence in 21.6%. Lower adherence was more frequent among patients in the lower age quartiles. No significant associations were observed between adherence and sex, disease duration, disease activity indices or treatment class.</p><p><strong>Conclusion: </strong>Medication adherence in BS appeared generally satisfactory, although a relevant proportion of patients reported suboptimal adherence. These findings confirm, in a BS-specific real-world setting, previously reported associations between age and medication adherence observed in chronic diseases. Routine adherence assessment may help identify patients at higher risk of poor treatment compliance and support personalised management strategies.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13386071/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148536739","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}
引用次数: 0
GLP-1 receptor agonists in systemic lupus erythematosus: a scoping review. 系统性红斑狼疮的GLP-1受体激动剂:范围综述。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-16 DOI: 10.1136/rmdopen-2026-007037
Robin Sia, Ee Lynn Ting, Suet-Wan Choy, Shereen Oon
{"title":"GLP-1 receptor agonists in systemic lupus erythematosus: a scoping review.","authors":"Robin Sia, Ee Lynn Ting, Suet-Wan Choy, Shereen Oon","doi":"10.1136/rmdopen-2026-007037","DOIUrl":"10.1136/rmdopen-2026-007037","url":null,"abstract":"<p><strong>Objective: </strong>Glucagon-like peptide-1 receptor agonists (GLP-1 RAs) are increasingly recognised for benefits beyond glycaemic control, including anti-inflammatory, anti-atherogenic and potential immunomodulatory effects. Given the high burden of metabolic dysfunction, accelerated cardiovascular disease and chronic systemic inflammation in systemic lupus erythematosus (SLE), significant interest has emerged regarding their therapeutic role in this population.</p><p><strong>Methods: </strong>We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-guided scoping review of PubMed, Embase, Scopus and the Cochrane Library from database inception to April 2026, including peer-reviewed clinical studies, case series, case reports and relevant research evaluating GLP-1 RAs in SLE while excluding conference abstracts and unpublished material.</p><p><strong>Results: </strong>Fourteen studies were included, comprising narrative reviews, mechanistic analyses, editorials and case reports. Across these studies, GLP-1 RAs demonstrated consistent anti-inflammatory, metabolic and endothelial effects relevant to SLE, with mechanistic data suggesting reductions in cytokine signalling, oxidative stress and macrophage activation. Current evidence specific to SLE is largely derived from small observational cohorts, retrospective analyses and isolated case reports, while much of the supportive anti-inflammatory and immunomodulatory data originate from preclinical studies or extrapolated from the broader rheumatic disease populations. GLP-1 RAs appear to have an acceptable safety profile in autoimmune populations, although drug-induced lupus was reported in two case studies.</p><p><strong>Conclusion: </strong>Collectively, findings from the included studies suggest that GLP-1 RAs may provide metabolic and anti-inflammatory benefits in SLE with an acceptable safety profile; however, clinical data specific to SLE remain limited and large high-quality prospective clinical trials are required to refine therapeutic indications, optimal patient selection and immunological effects.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13384179/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148472550","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}
引用次数: 0
Beyond Resnick and Niwayama: a structured narrative literature review on the clinical and imaging phenotypes of diffuse idiopathic skeletal hyperostosis (DISH). 超越Resnick和Niwayama:弥漫性特发性骨骼肥厚症(DISH)的临床和影像学表型的结构化叙事文献综述。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-16 DOI: 10.1136/rmdopen-2026-006809
Amir Bieber, David Kiefer, Fabiola Atzeni, Iris Eshed, Ana Rita Couto, Mariela Geneva-Popova, Netanja I Harlianto, Shay Brikman, Nicola Pappone, Pasquale Ambrosino, Giuseppe Scalise, Jácome Bruges Armas, Piercarlo Sarzi-Puttini, Jorrit Jan Verlaan, Muhammad Asim Khan, Reuven Mader, Xenofon Baraliakos
{"title":"Beyond Resnick and Niwayama: a structured narrative literature review on the clinical and imaging phenotypes of diffuse idiopathic skeletal hyperostosis (DISH).","authors":"Amir Bieber, David Kiefer, Fabiola Atzeni, Iris Eshed, Ana Rita Couto, Mariela Geneva-Popova, Netanja I Harlianto, Shay Brikman, Nicola Pappone, Pasquale Ambrosino, Giuseppe Scalise, Jácome Bruges Armas, Piercarlo Sarzi-Puttini, Jorrit Jan Verlaan, Muhammad Asim Khan, Reuven Mader, Xenofon Baraliakos","doi":"10.1136/rmdopen-2026-006809","DOIUrl":"10.1136/rmdopen-2026-006809","url":null,"abstract":"<p><p>Since 1975, the Resnick and Niwayama classification criteria have served as the standard framework for defining diffuse idiopathic skeletal hyperostosis (DISH) in research. These criteria predominantly identify advanced disease. Accumulating evidence towards an updated definition of DISH is warranted. In this review we aimed to re-evaluate the definition of DISH in light of contemporary evidence and identify elements that may inform future classification criteria.A literature review was conducted of studies published between 1975 and 2025. Data extraction targeted imaging features, including site-specific bone formation and pre-DISH characteristics, as well as clinical features, functional outcomes and available markers. Findings were qualitatively synthesised.119 studies were included. Imaging data indicated that degenerative spinal disease alone is insufficient to exclude DISH. Bony bridges most commonly affect the thoracic spine, though involvement of the cervical and lumbar regions is well documented. Disease progression is typically slow, and frequent entheseal involvement of pelvic, chest wall and peripheral sites. Epidemiological data demonstrate a rising prevalence with age and a male predominance. DISH shows strong association with metabolic syndrome, type 2 diabetes, elevated body mass index, raised systolic blood pressure, dyslipidaemia, hyperuricaemia and cardiovascular disease. Inflammatory markers are generally within normal ranges. Evidence regarding physical impairment remains mixed, with some studies reporting reduction in spinal mobility and chest expansion alongside broader functional limitations.This review moves beyond the Resnick and Niwayama criteria to offer an updated framework to support the development of future DISH classification criteria, particularly to earlier disease stages.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13384172/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148472625","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}
引用次数: 0
Time to diagnosis in large vessel vasculitis: insights from a retrospective study at a tertiary rheumatology centre. 大血管炎的诊断时间:来自三级风湿病中心回顾性研究的见解。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-15 DOI: 10.1136/rmdopen-2026-006782
Anna Kernder, Pauline Bussmann, Uta Kiltz, Hilal Kavruk, Philipp Sewerin, Johanna Mucke, Diana Vossen, David Kiefer, Ioana Andreica, Johanna Heuser, Judith Erkenberg, Panagiotis Ermeidis, Ralph Brinks, Xenofon Baraliakos
{"title":"Time to diagnosis in large vessel vasculitis: insights from a retrospective study at a tertiary rheumatology centre.","authors":"Anna Kernder, Pauline Bussmann, Uta Kiltz, Hilal Kavruk, Philipp Sewerin, Johanna Mucke, Diana Vossen, David Kiefer, Ioana Andreica, Johanna Heuser, Judith Erkenberg, Panagiotis Ermeidis, Ralph Brinks, Xenofon Baraliakos","doi":"10.1136/rmdopen-2026-006782","DOIUrl":"10.1136/rmdopen-2026-006782","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the diagnostic pathway of patients affected by large vessel vasculitis (LVV) and to determine factors associated with the time to diagnosis including referring and previously consulted medical specialties.</p><p><strong>Methods: </strong>This retrospective single-centre study enrolled patients diagnosed with giant cell arteritis (GCA) and Takayasu arteritis (TAK) between January 2014 and May 2025. Clinical, laboratory and referral data were retrieved from medical records. Time to diagnosis was defined as the interval between the onset of first LVV-related symptoms and the confirmed diagnosis. Factors associated with diagnostic delay were analysed by multivariate Cox regression. Temporal differences were visualised using Kaplan-Meier curves.</p><p><strong>Results: </strong>In total, 501 patients were included (GCA n=488; TAK n=13). Compared with GCA (89 days (IQR 31-184)), the median time to diagnosis for TAK (245 days (IQR 31-1308)) was longer. Presentation with typical cranial symptoms was associated with a shorter diagnostic interval (HR 1.27, p<i>=0.022</i>), involvement of the aorta and its major branches was linked to prolonged delay (HR 0.64, p<<i>0.001</i>). Prior medical consultations by rheumatologists were associated with longer diagnostic delay (HR 0.73, p=<i>0.004</i>), whereas referrals by ophthalmologists (HR 2.40, p=<i>0.001</i>) and cardiologists (HR 3.81, p=<i>0.008</i>) were associated with earlier diagnosis.</p><p><strong>Conclusion: </strong>Diagnostic delay varies by subtype and clinical presentation. Time to diagnosis was longer in TAK compared with GCA, whereas typical cranial symptoms facilitate earlier diagnosis. Increasing awareness is crucial to minimise diagnostic delay and prevent organ damage.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13374444/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456100","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}
引用次数: 0
Serum IgA autoantibody profiling in systemic lupus erythematosus reveals associations with disease phenotypes. 系统性红斑狼疮的血清IgA自身抗体分析揭示了与疾病表型的关联。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-15 DOI: 10.1136/rmdopen-2026-006811
Laura E Eades, Artemis Papadaki, Katie Lennard, Alberta Y Hoi, Rangi Kandane-Rathnayake, Eric F Morand, William A Figgett, Fabien B Vincent
{"title":"Serum IgA autoantibody profiling in systemic lupus erythematosus reveals associations with disease phenotypes.","authors":"Laura E Eades, Artemis Papadaki, Katie Lennard, Alberta Y Hoi, Rangi Kandane-Rathnayake, Eric F Morand, William A Figgett, Fabien B Vincent","doi":"10.1136/rmdopen-2026-006811","DOIUrl":"10.1136/rmdopen-2026-006811","url":null,"abstract":"<p><strong>Background: </strong>Knowledge of the role of IgA isotype autoantibodies in systemic lupus erythematosus (SLE) is lacking. We aimed to explore associations between serum IgA autoantibody profiles and SLE clinical phenotypes using an unbiased approach.</p><p><strong>Methods: </strong>1613 serum IgA autoantibodies were quantified in 60 patients with SLE using the i-Ome protein array, customised to also detect antibodies against three interferons (IFNα<sub>1</sub>, IFNω and IFNγ). Using clustering analysis, patients were grouped according to IgA autoantibody profiles; associations between clusters and clinical features were assessed using multinomial logistic regression. Associations between individual autoantibodies and clinical features were assessed via differential abundance analysis and supervised learning.</p><p><strong>Results: </strong>Five patient clusters were identified; patients in cluster 4, characterised by antitransforming acidic coiled-coil-containing protein 1 (anti-TACC1) and anti-cAMP-dependent protein kinase type I-alpha regulatory subunit (anti-PRKAR1A) IgA profiles, were significantly more likely to be of Asian ethnicity and have lupus nephritis than those in clusters 1 and 3. Patients in cluster 4 and those in cluster 3, characterised by antiangiomotin-like protein 2 (anti-AMOTL2) IgA profiles, were significantly more likely to use immunosuppression than those in cluster 2. Analysis of individual autoantibodies revealed a significant association between antisignal transducing adaptor molecule 2 (anti-STAM2) and haematological disease activity. Targeted analysis demonstrated associations between several autoantibodies to IFN-associated proteins and SLE phenotypes, including ethnicity, disease activity, immunosuppression and organ damage.</p><p><strong>Conclusions: </strong>Clustering according to IgA autoantibody profiles suggests the presence of a subgroup of patients with SLE associated with Asian ethnicity, lupus nephritis and immunosuppression use. Additionally, a novel association between anti-STAM2 IgA and haematological disease was discovered. These findings demonstrate the pathophysiological relevance of IgA autoantibodies in SLE and suggest their potential as SLE biomarkers.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13374403/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456757","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}
引用次数: 0
Expert viewpoint on endpoints in systemic sclerosis: current and future outlook. 系统性硬化症终点的专家观点:当前和未来展望。
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-15 DOI: 10.1136/rmdopen-2026-006784
Anna-Maria Hoffmann-Vold, Oliver Distler, Francesco Del Galdo, Jörg Hw Distler, Yannick Allanore, Elana J Bernstein, Christopher P Denton, Masataka Kuwana, Marco Matucci-Cerinic, Margarida Alves, Vanessa Smith
{"title":"Expert viewpoint on endpoints in systemic sclerosis: current and future outlook.","authors":"Anna-Maria Hoffmann-Vold, Oliver Distler, Francesco Del Galdo, Jörg Hw Distler, Yannick Allanore, Elana J Bernstein, Christopher P Denton, Masataka Kuwana, Marco Matucci-Cerinic, Margarida Alves, Vanessa Smith","doi":"10.1136/rmdopen-2026-006784","DOIUrl":"10.1136/rmdopen-2026-006784","url":null,"abstract":"<p><p>Systemic sclerosis (SSc) is a rare, heterogeneous, systemic autoimmune rheumatic disease characterised by vasculopathy, immune dysregulation and fibrosis affecting multiple organ systems. The complexity and variability of SSc manifestations pose significant challenges for clinical trial design and endpoint selection. Historically, three types of trial concepts have emerged: studies focused on a single organ; studies suggesting disease modification by combining a primary single-organ endpoint with supportive secondary endpoints across other systems, and studies explicitly designed to demonstrate disease modification through combined endpoints.This expert review explores the current landscape and future directions of clinical endpoints in SSc trials. It highlights the growing importance of patient-reported outcomes, such as the Health Assessment Questionnaire-Disability Index and ScleroID, which provide valuable insights into patient experiences. The review also discusses the emergence of candidate composite endpoints, including the revised Composite Response Index in Systemic Sclerosis, which integrates clinical, functional and patient-centred measures to better reflect treatment efficacy.Despite progress, significant unmet needs remain. Many endpoints assess damage at advanced stages, underscoring the need for earlier indicators of disease progression. The review advocates for incorporating time-to-event analyses, imaging and biomarker-based assessments to enhance sensitivity and relevance. It also emphasises the importance of refining composite endpoints to include continuous measures and broader domains such as vascular and gastrointestinal involvement. These efforts aim to improve the design and interpretability of SSc clinical trials, ultimately enhancing therapeutic development and patient outcomes.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13374462/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456647","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}
引用次数: 0
Prevalence and risk factors for high-risk human papillomavirus infection in patients with immune-mediated inflammatory diseases: a prospective study. 免疫介导炎症性疾病患者高危人乳头瘤病毒感染的患病率和危险因素:一项前瞻性研究
IF 5.3 2区 医学
RMD Open Pub Date : 2026-07-15 DOI: 10.1136/rmdopen-2026-006955
Tiphaine Goulenok, Charlotte Sierra, Aline Marie Florence, Clementine Gonthier, Margot Bucau, Thomas Papo, Arthur Mageau, Valentine Marie Ferré, Karim Sacre
{"title":"Prevalence and risk factors for high-risk human papillomavirus infection in patients with immune-mediated inflammatory diseases: a prospective study.","authors":"Tiphaine Goulenok, Charlotte Sierra, Aline Marie Florence, Clementine Gonthier, Margot Bucau, Thomas Papo, Arthur Mageau, Valentine Marie Ferré, Karim Sacre","doi":"10.1136/rmdopen-2026-006955","DOIUrl":"10.1136/rmdopen-2026-006955","url":null,"abstract":"<p><strong>Objective: </strong>Women with immune-mediated inflammatory diseases (IMID) are at increased risk of cervical cancer. We aimed to determine the prevalence of carcinogenic high-risk human papillomavirus (HR-HPV) infection and identify factors associated with infection in women with IMID.</p><p><strong>Methods: </strong>We conducted a prospective, single-centre study at a French university hospital. We assessed compliance with cervical cancer screening (CCS) recommendations among all patients aged 30-65 years who attended follow-up visits between March 2024 and May 2025. HPV screening with vaginal self-sampling (VSS) was offered to all women not up to date with CCS. The primary outcome was HR-HPV infection prevalence.</p><p><strong>Results: </strong>Among the 485 patients eligible for CCS, 296 were not up to date with CCS and were therefore eligible for VSS. Of these, 276 (mean age: 48±10 years) consented to participate and were included in the study. HR-HPV was detected in 55 patients (20%, n=55/276), among whom 35% (n=19/55) harboured multiple HR-HPV infections (≥2 types). In multivariable analysis, age at first sexual intercourse (OR (95% CI) 0.85 (0.73 to 0.96)) and number of lifetime sexual partners (OR 1.19 (1.05 to 1.38)) were associated with HR-HPV infection. In exploratory multivariable analyses, longer duration of biological therapy was associated with multiple HR-HPV infections (OR 1.57 (0.99 to 2.85)).</p><p><strong>Conclusions: </strong>The prevalence of HR-HPV infection in patients with IMID who are not up to date with CCS is 20%. Behavioural factors were associated with HR-HPV infection, while treatment-related findings regarding multiple HR-HPV infections were exploratory and warrant confirmation in larger prospective studies.</p><p><strong>Trial registration number: </strong>NCT06302725.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"12 3","pages":""},"PeriodicalIF":5.3,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13374457/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456765","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}
引用次数: 0
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