RMD Open最新文献

{"title":"Autoantibody clusters in rheumatoid arthritis are not driven by antigen specificity or isotype.","authors":"Anouk G van Mourik, Linda Johansson, Tineke J van Wesemael, Marc P Maurits, Heidi Kokkonen, Johan Rönnelid, Rachel Knevel, René E M Toes, Solbritt Rantapää-Dahlqvist, Diane van der Woude","doi":"10.1136/rmdopen-2024-005291","DOIUrl":"https://doi.org/10.1136/rmdopen-2024-005291","url":null,"abstract":"<p><strong>Objective: </strong>Autoantibodies are a key feature of rheumatoid arthritis (RA). They can be detected years before disease onset, but it is unknown if there is any pattern in the co-occurrence of antigen recognition or isotype profiles. A common signature could point to a unique initial trigger for autoantibody development. Therefore, we sought to determine if there is a pattern in antigen or isotype reactivity in pre-symptomatic cases and established RA.</p><p><strong>Methods: </strong>One pre-symptomatic cohort and one RA cohort were analysed for the co-occurrence of different isotypes of anti-modified protein antibodies (AMPA) and rheumatoid factor (RF). Patterns in autoantibody levels were investigated with clustering. Additionally, total IgG was measured in 1- year follow-up sera of a representative subgroup of the RA cohort.</p><p><strong>Results: </strong>While especially anti-citrullinated protein antibodies (ACPA) IgG and RF IgA co-occurred with other autoantibodies, no specific patterns emerged. In both cohorts, clusters of autoantibody levels were not determined by particular antigen reactivities or isotype. However, clusters were driven by elevated levels of several different AMPA, with distinct AMPA high- and low-level clusters. A broad IgG autoantibody profile was not accompanied by high total IgG levels.</p><p><strong>Conclusion: </strong>Autoantibody clusters are most likely not driven by AMPA specificity or isotype profile, neither before nor at RA onset, but are instead determined by a broad variety of autoantibodies. This indicates that the triggers for autoantibody development in RA do not skew the response towards certain autoreactivities or isotypes but rather lead to a broad and diverse autoantibody repertoire reflecting continuous and ongoing immune activation.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12164635/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144294865","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":"Rising incidence of systemic autoimmune inflammatory rheumatic diseases during the COVID-19 pandemic: a geographical cohort study.","authors":"Enrico De Lorenzis, Paolo Parente, Salvatore Soldati, Andrea Barbara, Gerlando Natalello, Marina Davoli, Silvia Laura Bosello, Maria Antonietta D'Agostino, Mirko Di Martino","doi":"10.1136/rmdopen-2024-005227","DOIUrl":"https://doi.org/10.1136/rmdopen-2024-005227","url":null,"abstract":"<p><strong>Objective: </strong>The natural infection with SARS-CoV-2, or vaccination against it, has been postulated to directly contribute to an increase in the incidence of autoimmune inflammatory rheumatic diseases (AIIRDs). Conversely, preventive measures limiting access to healthcare services could have resulted in missed or delayed AIIRD diagnoses or have reduced the infection rate of any triggering infections. We aimed to define real-life trends in AIIRD diagnoses from the prepandemic period through 2023 in a large and geographically circumscribed population of 6.5 million inhabitants.</p><p><strong>Methods: </strong>AIIRDs' annual diagnosis rates from 2017 to 2023 were derived from the registration of disease-specific exemption codes of the resident population of Lazio, a highly populated region in central Italy. Incidence rate ratios (IRRs) were calculated to compare pandemic and average prepandemic rates (2017-2019). Poisson regression was used to define statistically significant changes.</p><p><strong>Results: </strong>A total of 16 254 AIIRD diagnoses were registered over the 7-year period. The average prepandemic incidence of AIIRDs was 4.81 per 10 000 inhabitants (95% CI 4.69 to 4.92). Compared with the prepandemic period, the diagnosis rate decreased in 2020 (IRR 0.68, 95% CI 0.64 to 0.72) but remained above prepandemic levels in 2021, 2022 and 2023. In 2023, there was a 22% increase in AIIRD incidence compared with prepandemic levels (IRR 1.22, 95% CI 1.17 to 1.28, p<0.001). This excess incidence was primarily driven by increases in both primary arthritides and systemic rheumatic diseases.</p><p><strong>Conclusions: </strong>We observed a temporary decline in diagnosis in 2020, followed by a substantial increase from 2021 to 2023. This trend (decline and increase) may be linked to COVID-19 infection or to the reduction and subsequently potential increase of other infectious triggers following the use of preventive measures, such as facial masks and social distancing.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12164633/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144294867","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":"Impact of enhanced pain knowledge on core outcomes in fibromyalgia patients with high self-reported pain education needs: a target trial emulation using the DANFIB registry.","authors":"Kirstine Amris, Robin Christensen, Eva Ejlersen Wæhrens, Pernille Hurup Duhn, Marius Henriksen","doi":"10.1136/rmdopen-2025-005576","DOIUrl":"https://doi.org/10.1136/rmdopen-2025-005576","url":null,"abstract":"<p><strong>Objective: </strong>Relationships between patient education and long-term clinical outcomes are complex. This study used real-world data to evaluate the impact of improved pain knowledge on clinical outcomes in fibromyalgia patients.</p><p><strong>Methods: </strong>Prospectively collected registry-based observational data were analysed to emulate a randomised clinical trial. Study participants were diagnosed with fibromyalgia, had a high need to learn about pain (Numeric Rating Scale >7), had attended a 2-day therapeutic educational programme and re-rated their learning needs after the programme. A good educational outcome was defined as a re-rating score <5, while a poor outcome was ≥5. The primary endpoint at 9 months was the overall impact of fibromyalgia, measured by the Fibromyalgia Impact Questionnaire Revised (FIQR) impact subscale.</p><p><strong>Results: </strong>The eligible cohort comprised 450 participants. The intention-to-treat from population included 121 participants (26.9%) with a good educational outcome and 329 participants (73.1%) with a poor educational outcome (comparator group). Missing outcome data were handled implicitly by the repeated measures linear mixed models, assuming data are missing at random.At the 9-month endpoint, the fully adjusted FIQR impact subscale score was lower in the good educational outcome group (8.0 (95% CI 7.3 to 8.7)) compared with the poor educational outcome group (9.6 (95% CI 9.0 to 10.2)), indicating a better clinical outcome for those with a good educational outcome. The model-estimated between-group difference was -1.6 (95% CI -2.5 to -0.7; p=0.0006) FIQR impact subscale units.</p><p><strong>Conclusions: </strong>This study suggests that achieving pain educational learning objectives leads to better clinical outcomes in fibromyalgia patients, supporting the integration of pain education into patient programmes.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12164631/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144294866","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":"Combination therapy of rituximab and mycophenolate in patients with systemic sclerosis and primary cardiac involvement refractory to cyclophosphamide: a retrospective exploratory analysis of 10 cases.","authors":"El-Baraa Adjailia, Hanna Grasshoff, Susanne Schinke, Konstantinos Fourlakis, Sebastian T Jendrek, Peter Lamprecht, Gabriela Riemekasten, Jens Y Humrich","doi":"10.1136/rmdopen-2025-005493","DOIUrl":"10.1136/rmdopen-2025-005493","url":null,"abstract":"<p><p>Despite the high mortality risk, no specific treatment options for cardiac manifestations in systemic sclerosis (SSc) currently exist. We performed a retrospective medical records analysis at our centre to explore the therapeutic effects of a combination therapy with rituximab (RTX) and mycophenolate (MMF) in 10 patients with SSc-related primary cardiac involvement refractory to previous primary treatment with cyclophosphamide (CP).SSc-related primary cardiac involvement was defined as the presence of troponin T elevation and of at least one of the following cardiac manifestations: right or left ventricular systolic or diastolic dysfunction, myocarditis, pericarditis, heart blocks or ventricular arrhythmias. Patients who had worsening or persistence of cardiac involvement after CP pulse therapy received a combination therapy of RTX (1000 mg every 3-6 months) and MMF (up to 3000 mg/day). Cardiac outcomes were evaluated during a 6-12-month follow-up period.Following the initiation of the combination therapy, consistent decreases in plasma levels of troponin T were observed in all patients (p=0.0020). Corresponding to this, left ventricular ejection fraction (LVEF) improved between 3% and 23% in five of the six patients with reduced LVEF, the rate of ventricular extrasystoles declined in all assessable patients (n=6, p=0.0313) and N-terminal pro hormone of brain natriuretic peptide decreased in six of the nine patients with elevated levels. Significant reductions in modified Rodnan skin score were also observed (p=0.0020). RTX/MMF combination was generally well tolerated. In the long-term follow-up period of up to 6 years, seven serious adverse events consisting of five infections and two fatal events were recorded.Our findings suggest that combination therapy with RTX and MMF may be an effective approach for improving refractory cardiac manifestations in patients with SSc. Controlled and prospective studies are required to further substantiate these encouraging observations and to prove the long-term safety of RTX/MMF combination.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161380/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275863","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":"Age at diagnosis as a prognostic factor in selected categories of juvenile idiopathic arthritis.","authors":"Anna-Kaisa Tuomi, Katariina Rebane, Ellen Arnstad, Lillemor Berntson, Anders Fasth, Mia Glerup, Troels Herlin, Hannu Kautiainen, Ellen Berit Nordal, Suvi Peltoniemi, Marite Rygg, Veronika Rypdal, Marek Zak, Kristiina Aalto","doi":"10.1136/rmdopen-2024-005369","DOIUrl":"10.1136/rmdopen-2024-005369","url":null,"abstract":"<p><strong>Introduction: </strong>The age at the onset of juvenile idiopathic arthritis (JIA) can influence the trajectory of the disease. We aimed to clarify how age at the visit 6 months after the onset as a continuous variable affects long-term remission of JIA.</p><p><strong>Methods: </strong>This study investigated 358 patients from the Nordic JIA cohort study. Age at diagnosis was analysed continuously. Three age groups were studied: under 3 years, 3-5 years and over 6 years of age. JIA was categorised as oligoarthritis, seronegative polyarthritis and others (enthesitis-related, psoriatic and undifferentiated arthritis). Clinical data, collected at 6 months after the onset of symptoms, included information about disease activity, uveitis, laboratory test values and medication. The outcomes assessed 17.5 years after diagnosis included remission, health-related quality of life (HRQoL), and functional ability.</p><p><strong>Results: </strong>The majority of patients with oligoarthritis and polyarthritis were diagnosed before age six, compared with 29% in the group of others. In the oligoarthritis group, predictors of remission included age at diagnosis, male gender, the Juvenile Arthritis Disease Activity Score-71 (JADAS71) and the absence of uveitis. In seronegative polyarthritis, predictors of remission were age at diagnosis and JADAS71 score. In the oligoarthritis group, remission rates were highest in both genders when diagnosed <3 years of age. In the seronegative polyarthritis group, this was not true for female patients. Age at diagnosis had no significant effect on HRQoL or functional ability.</p><p><strong>Conclusions: </strong>Age at diagnosis in the oligoarthritis was inversely and in the seronegative polyarthritis positively associated with long-term remission in JIA, primarily in females.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161311/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275862","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":"Clinical utility of large language models for osteoporosis referral triage: a pilot study.","authors":"Sergio Del Vescovo, Pallavi Vij, Charlotte Casteleyn, Ilke Coskun Benlidayi, Muhamad Jasim, Tochukwu Adizie, Hem Sapkota, Srinivasan Venkatachalam, Latika Gupta, Vincenzo Venerito","doi":"10.1136/rmdopen-2025-005604","DOIUrl":"10.1136/rmdopen-2025-005604","url":null,"abstract":"","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161361/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144249381","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":"Glucocorticoid treatment in early rheumatoid arthritis is independently associated with increased PCSK9 levels: data from a randomised controlled trial.","authors":"Kristina Lend, Jos Wr Twisk, Nupur Kumar, Bas Dijkshoorn, Jon Lampa, Anna Rudin, Merete Lund Hetland, Till Uhlig, Dan Nordström, Mikkel Østergaard, Bjorn Gudbjornsson, Tuulikki Sokka-Isler, Gerdur Grondal, Kim Hørslev-Petersen, Michael T Nurmohamed, Johan Frostegård, Ronald F van Vollenhoven","doi":"10.1136/rmdopen-2024-005129","DOIUrl":"10.1136/rmdopen-2024-005129","url":null,"abstract":"<p><strong>Background: </strong>Rheumatoid arthritis elevates cardiovascular disease risk. Proprotein convertase subtilisin/kexin type 9 (PCSK9), a regulator of low-density lipoprotein (LDL) metabolism, increases LDL-receptor breakdown in the liver, which elevates LDL-cholesterol levels. In addition, PCSK9 has direct effects on thrombogenesis and atherosclerotic plaque formation.We aimed to investigate (1) the impact of glucocorticoids and biological disease-modifying antirheumatic drug (bDMARD) treatments on PCSK9 and LDL-cholesterol levels, (2) whether this influence is different when autoantibodies are present and (3) the association between PCSK9 and LDL cholesterol.</p><p><strong>Methods: </strong>In this post hoc analysis of the NORD-STAR trial, 296 newly diagnosed patients starting methotrexate with glucocorticoids, certolizumab pegol, abatacept or tocilizumab were included. Serum PCSK9 and LDL-cholesterol levels were measured at baseline and 24 weeks. Linear regression models were used to analyse the difference in PCSK9 and LDL cholesterol between glucocorticoid and bDMARD treatments at 24 weeks. In the second analysis, the interactions between the treatment groups and autoantibody status were added to the model.</p><p><strong>Results: </strong>After 24 weeks, PCSK9 levels were higher in the glucocorticoid group than in the combined bDMARD treatment group (-276.0 (95% CI -468.2 to -83.9)). When compared with the bDMARD treatment, these increases were more pronounced in autoantibody-positive patients. Changes in LDL cholesterol exhibited a pattern distinct from PCSK9, as it increased in all treatments.</p><p><strong>Conclusion: </strong>Glucocorticoid treatment was associated with increased PCSK9 levels after 24 weeks. When compared with the bDMARD treatments, these increases were more pronounced in rheumatoid factor, anticitrullinated protein antibody and antinuclear antibody-positive patients. Our data provide a potential mechanistic link between glucocorticoid treatment and cardiovascular disease.</p><p><strong>Funding: </strong>Inger Bendix Foundation for Medical Research.</p><p><strong>Trial registration number: </strong>EudraCT2011-004720-35, NCT01491815.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161332/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144249382","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":"Predictors of persistent active disease and the longitudinal development of organ damage, patient-reported outcomes and autoantibodies in long-term juvenile dermatomyositis.","authors":"Simon Girmai Berger, Kristin Schjander Berntsen, Kristine Risum, Henriette Schermacher Marstein, Ivar Sjaastad, Mathis Korseberg Stokke, Helga Sanner","doi":"10.1136/rmdopen-2025-005598","DOIUrl":"10.1136/rmdopen-2025-005598","url":null,"abstract":"<p><strong>Objective: </strong>To quantify longitudinal development of disease activity, organ damage, patient-reported outcomes, and myositis autoantibody profiles in patients with juvenile dermatomyositis (JDM) after long-term follow-up, and to identify predictors for persistent active disease.</p><p><strong>Methods: </strong>Forty patients (65% female) diagnosed with JDM were clinically examined at two different time points (visits 1 and 2). We assessed clinically inactive/active disease by the updated PRINTO criteria and the Juvenile DermatoMyositis Activity Index (JDMAI). Organ damage was evaluated by Myositis Damage Index (MDI) and physical function by Childhood Health Assessment Questionnaire (CHAQ/HAQ). Myositis autoantibodies were measured by myositis line immunoassay.</p><p><strong>Results: </strong>Median disease duration from symptom onset was 15.1 (2.0-34.6) at visit 1 and 21.7 (7.6-42.7) years at visit 2. At visit 2, active disease (PRINTO) was found in 53%, impaired physical function (CHAQ/HAQ>0) in 40%, organ damage (MDI_total≥1) in 95% and myositis specific or associated antibodies in 33% of patients. Disease activity (JDMAI) was low in 24%, moderate in 8% and high in 3% of patients. There were no significant differences in organ damage and disease activity between visits. Higher disease activity and organ damage at visit 1 predicted persistent active disease at visit 2.</p><p><strong>Conclusions: </strong>After a median of 21.7 years from symptom onset, the majority of JDM patients still had active disease. Higher organ damage and disease activity at the initial visit predicted persistent active disease at follow-up. These results underscore the chronic nature of JDM, emphasising the need for enhanced early and long-term management strategies to improve patient outcomes.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161408/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144249384","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":"Long-term maintenance of remission with spacing of rituximab infusions based on the individualised patient risk profile in ANCA-associated vasculitis: a pilot study.","authors":"Alice Bartoletti, Alessandra Milanesi, Sara Monti, Carlomaurizio Montecucco, Paolo Delvino","doi":"10.1136/rmdopen-2025-005504","DOIUrl":"10.1136/rmdopen-2025-005504","url":null,"abstract":"","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161294/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144249383","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":"Cluster analysis identifies three clinical patterns of patients with systemic autoimmune diseases and anti-Ku antibodies.","authors":"Marie Robert, Yann Nguyen, Yves Allenbach, Karim Sacre, Benjamin Terrier, Raphael Borie, Yurdagul Uzunhan, Zahir Amoura, Céline Comparon, Philippe Dieudé, Véronique Le Guern, Capucine Morelot-Panzini, Marc Humbert, Olivier Sitbon, Cécile Goujard, Brigitte Bader-Meunier, Bruno Fautrel, Pascale Chretien, 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, Francois Chasset, Gaétane Nocturne, Xavier Mariette, Samuel Bitoun, Raphaele Seror","doi":"10.1136/rmdopen-2024-005191","DOIUrl":"10.1136/rmdopen-2024-005191","url":null,"abstract":"<p><strong>Objective: </strong>To determine distinct patterns of patients with autoimmune diseases harbouring anti-Ku antibodies and their respective prognosis.</p><p><strong>Methods: </strong>Anti-Ku-positive patients were retrieved through four immunology departments. Clusters were derived from unsupervised multiple correspondence analysis, not including the disease's diagnosis, followed by hierarchical clustering. Baseline characteristics and risk of disease progression, defined as a composite of new organ involvement or the need for new immunosuppressants, were compared across the retrieved clusters.</p><p><strong>Results: </strong>Among 154 anti-Ku-positive patients, three clusters were identified. At disease's onset, all patients included in cluster 1 (n=42/154, 27%) had muscle involvement, 34% displayed cardiac manifestations. Inflammatory myopathies (n=35/42, 83%) and/or systemic sclerosis (n=17/42, 40%) were the most frequent diagnoses. Cluster 2 (n=69/154, 45%) included the lowest proportion of women (68% vs 83% and 84% in clusters 1 and 3), 54% of patients had lung involvement, and 25% fulfilled Sjögren's disease criteria. Cluster 3 (n=43/154, 28%) included younger patients (median age 25 years), with 79% of them fulfilling systemic lupus erythematosus criteria. These three clusters have distinct outcomes (p=0.001): cluster 1 developed lung involvement and displayed the higher risk of disease progression, cluster 2 was prone to myositis development and cluster 3 developed various clinical manifestations. The proportion of patients with heart involvement doubled over time in all clusters, with a majority of myocarditis in cluster 1, pulmonary hypertension in cluster 2 and pericarditis in cluster 3.</p><p><strong>Conclusion: </strong>Three distinct groups of anti-Ku-positive patients were identified; cardiac involvement should be carefully tracked throughout the follow-up in all of them.</p>","PeriodicalId":21396,"journal":{"name":"RMD Open","volume":"11 2","pages":""},"PeriodicalIF":5.1,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12142139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144235030","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}