{"title":"Variability in phenotype clusters of Behçet’s syndrome: A systematic review","authors":"Betul Macit , Sinem Nihal Esatoglu , Kevser Akyuz-Yesilyurt , Gulen Hatemi","doi":"10.1016/j.semarthrit.2025.152744","DOIUrl":"10.1016/j.semarthrit.2025.152744","url":null,"abstract":"<div><h3>Background</h3><div>Behçet’s syndrome (BS) is a multisystem vasculitis, and distinct clinical phenotypes with clustering of certain organ manifestations were proposed. However, studies from different cohorts have shown variability in the defined phenotypes. This was attributed to geographic and ethnic differences, but different studies from the same country have also shown variability in phenotype clusters. We aimed to explore the variability in clinical phenotype clustering across different countries and cohorts and possible reasons for these.</div></div><div><h3>Methods</h3><div>An electronic search was carried out in PubMed, EMBASE, and Cochrane Library for studies that assessed phenotype clusters in BS cohorts. Two reviewers independently performed the screening of titles, abstracts, and full texts using Covidence.</div></div><div><h3>Results</h3><div>A total of 15 studies that assessed 17 different cohorts were identified. Several differences were identified in the clusters that were reported in these cohorts. Factors that were identified by this systematic review as possible causes of these differences were study design, statistical analysis method (hierarchical cluster analysis vs. factor analysis), patient population (pediatric vs. adult), setting, diagnostic/classification criteria (International Study Group vs. International Criteria for Behçet’s Disease), disease duration, the definition of organ involvement (such as including cerebral sinus thrombosis in nervous system or vascular involvement), ascertainment of manifestations (such as gastrointestinal involvement confirmed by endoscopy or not), and time component for clustering of manifestations.</div></div><div><h3>Conclusion</h3><div>There is important variability in the phenotype clusters that are reported in different studies and this variability seems to stem from methodologic differences between the studies.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152744"},"PeriodicalIF":4.6,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143947527","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}
Olga Rusinovich-Lovgach , Zulema Plaza , Mónica Fernández Castro , Jose Rosas-Gómez de Salazar , Victot Manuel Martínez Taboada , Alejandro Olive , Raúl Menor Almagro , Belen Serrano Benavente , Judit Font Urgelles , Angel Garcia-Aparicio , Sara Manrique-Arija , Jesús Alberto Garcia Vadillo , Ruth Lopez-Gonzalez , Javier Narvaez García , Mª Beatriz Rodriguez Lozano , Carlos Galisteo , Jorge Juan Gonzalez Martin , Paloma Vela Casasempere , Elena Rabadán , Antonio Naranjo , José Luis Andréu Sánchez
{"title":"Cancer risk in Sjögren’s disease: A longitudinal cohort study on incidence, predictors, and mortality impact","authors":"Olga Rusinovich-Lovgach , Zulema Plaza , Mónica Fernández Castro , Jose Rosas-Gómez de Salazar , Victot Manuel Martínez Taboada , Alejandro Olive , Raúl Menor Almagro , Belen Serrano Benavente , Judit Font Urgelles , Angel Garcia-Aparicio , Sara Manrique-Arija , Jesús Alberto Garcia Vadillo , Ruth Lopez-Gonzalez , Javier Narvaez García , Mª Beatriz Rodriguez Lozano , Carlos Galisteo , Jorge Juan Gonzalez Martin , Paloma Vela Casasempere , Elena Rabadán , Antonio Naranjo , José Luis Andréu Sánchez","doi":"10.1016/j.semarthrit.2025.152743","DOIUrl":"10.1016/j.semarthrit.2025.152743","url":null,"abstract":"<div><h3>Objectives</h3><div>This study aimed to evaluate standardized incidence ratios (SIRs) of overall malignancies, hematologic malignancies and solid tumors in patients with Sjögren’s disease (SjD) compared to the general population. Furthermore, it sought to identify independent predictors of malignancy and quantify the impact of cancer on mortality.</div></div><div><h3>Methods</h3><div>This prospective, multicenter study included 314 patients clinically diagnosed with SjD and fulfilling 2002 American-European Consensus Group criteria, with a median follow-up of 9.5 years. Clinical, demographic, and serological data were collected, along with malignancy incidence and mortality outcomes. SIRs were calculated using GLOBOCAN data. Multivariate Cox regression identified malignancy predictors. The relative risk (RR) of death and the etiologic fraction in exposed individuals (EFE) assessed cancer-related mortality.</div></div><div><h3>Results</h3><div>A total of 22 malignancies (7.01%) were identified, including 11 hematologic malignancies (50%) and 11 solid tumors (50%). The overall cancer risk was increased (SIR: 1.68, 95% CI: 1.68–1.69), with a substantially higher risk for hematologic malignancies (SIR: 3.55, 95% CI: 3.54–3.56) and a moderate increase for solid tumors (SIR: 1.54, 95% CI: 1.53–1.55). All hematologic malignancies were non-Hodgkin lymphomas (NHL). Independent predictors of malignancy included older age, smoking, lymphadenopathy, splenomegaly, and cryoglobulinemia. Cancer was responsible for 23.8% of deaths (RR: 2.21, EFE: 55%).</div></div><div><h3>Conclusions</h3><div>Patients with SjD have an elevated malignancy risk, mainly driven by NHL, while solid tumor risk remains modest. Malignancy was a significant contributor to mortality. These findings underscore the need for better risk stratification and targeted surveillance in high-risk SjD patients for early detection and intervention.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152743"},"PeriodicalIF":4.6,"publicationDate":"2025-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143929509","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}
J.A. van der Pol , E.G. Brilman , P.H.P. de Jong , A.E.A.M. Weel , L.R. Lard , E.T. Molenaar , T.W.J. Huizinga , S.A. Bergstra , C.F. Allaart
{"title":"A comparison of short-term efficacy and toxicity of 2 glucocorticoid bridging strategies in 2 clinical trials in early rheumatoid and undifferentiated arthritis","authors":"J.A. van der Pol , E.G. Brilman , P.H.P. de Jong , A.E.A.M. Weel , L.R. Lard , E.T. Molenaar , T.W.J. Huizinga , S.A. Bergstra , C.F. Allaart","doi":"10.1016/j.semarthrit.2025.152748","DOIUrl":"10.1016/j.semarthrit.2025.152748","url":null,"abstract":"<div><h3>Objectives</h3><div>To compare short-term outcomes of initial methotrexate therapy with higher or lower-dose glucocorticoid (GC) bridging in patients with early rheumatoid or undifferentiated arthritis.</div></div><div><h3>Methods</h3><div>We compared two trials: a ‘higher-dose GC’-study starting with methotrexate and 60 mg/day prednisone, tapered in 7 weeks to 7.5 mg/day (IMPROVED trial) and a ‘lower-dose GC’-study, starting with methotrexate and prednisone 15 mg/day tapered in 10 weeks to nil (arm C of the tREACH trial). After multiple imputation, we compared the DAS and HAQ, rates of DAS-remission (DAS<1.6) and low disease activity (DAS≤2.4) at the first follow-up visit after 3 to 4 months with linear and logistic regression models, adjusted for baseline DAS/HAQ, age, gender, symptom duration, ACPA positivity, BMI and damage.</div></div><div><h3>Results</h3><div>Baseline symptom duration, DAS and HAQ were comparable, but more patients in the lower-dose GC-study arm C fulfilled the 2010 criteria for RA. After correction for confounders, patients in the lower-dose GC-study arm C had a significantly higher DAS (0.62 higher (95 % CI 0.43; 0.80) and HAQ (0.28 higher (95 % CI 0.17; 0.39) at the first follow-up visit compared to patients in the higher-dose GC-study, and less often DAS-remission (63.4 % versus 28.9 %) and low disease activity (80.6 % versus 55.7 %). Fewer adverse events were reported in the higher-dose GC-study.</div></div><div><h3>Conclusion</h3><div>In patients with early RA or UA, a study with higher dosed glucocorticoids as part of initial treatment was associated with significantly better early clinical outcomes compared to a study with lower dosed glucocorticoids, and fewer early side effects. These results should be interpreted with caution due to risk of bias when comparing two distinct clinical trials instead of performing one trial.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152748"},"PeriodicalIF":4.6,"publicationDate":"2025-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143942370","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}
Barbara Russo , Iulia-Simona Chirică , Delphine Sophie Courvoisier , Michele Iudici
{"title":"Trial-level factors affecting accrual rate of systemic sclerosis randomized clinical trials","authors":"Barbara Russo , Iulia-Simona Chirică , Delphine Sophie Courvoisier , Michele Iudici","doi":"10.1016/j.semarthrit.2025.152749","DOIUrl":"10.1016/j.semarthrit.2025.152749","url":null,"abstract":"<div><h3>Objectives</h3><div>To estimate the average time to complete patient enrollment and identify factors associated with accrual rates in systemic sclerosis (SSc) randomized controlled trials (RCTs).</div></div><div><h3>Methods</h3><div>We searched published SSc-RCTs indexed in PubMed from 2000 to 2024, selecting those with recruitment completed before the COVID-19 pandemic. We recorded key trial features (country, phase, randomization ratio, intervention, blinding, funding source, outcome type) and enrollment year(s). We measured enrollment duration and accrual rate (participants per month). A multivariable negative binomial generalized linear model was used to identify factors associated with accrual rate.</div></div><div><h3>Results</h3><div>We included 80 studies, mostly single-country (75.0 %) and industry-funded (57.5 %), mainly recruiting in Europe (36.2 %) and North America (22.5 %). In 65 % of studies, both limited and diffuse SSc patients were enrolled. The median sample size was 40.5 patients, with 20 % of RCTs enrolling ≥100 patients. The median recruitment time was 15 months (IQR 9.9 – 30.0), with a median accrual rate of 3.1 (IQR 1.6 - 5.5) participants per month. Recruitment rates varied over time, with faster accrual early in the 2000s and after 2012, and a slower period in between. Multivariable analysis showed that accrual rate was positively associated with skewed randomization, blinding, non-industry funding, international recruitment, and inclusion of both SSc subsets, especially compared to studies involving only dcSSc patients.</div></div><div><h3>Conclusions</h3><div>Recruiting SSc patients for RCTs has been challenging, with generally slow accrual over the past 20 years and no significant improvement over time.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152749"},"PeriodicalIF":4.6,"publicationDate":"2025-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143929510","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}
Laetitia Perronne , Marie Binvignat , Nathan Foulquier , Alain Saraux , Jean Denis Laredo , Constance de Margerie-Mellon , Laure Fournier , Jérémie Sellam
{"title":"Algorithmic approaches in hand imaging for rheumatic musculoskeletal diseases: A systematic literature review","authors":"Laetitia Perronne , Marie Binvignat , Nathan Foulquier , Alain Saraux , Jean Denis Laredo , Constance de Margerie-Mellon , Laure Fournier , Jérémie Sellam","doi":"10.1016/j.semarthrit.2025.152750","DOIUrl":"10.1016/j.semarthrit.2025.152750","url":null,"abstract":"<div><h3>Objective</h3><div>This systematic literature review provides a comprehensive overview of the use of machine learning (ML) in hand imaging of rheumatic musculoskeletal diseases (RMDs). The review evaluates ML algorithms, imaging modalities, patient populations, validation methods, and areas for improvement.</div></div><div><h3>Methods</h3><div>The review was conducted following PRISMA guidelines and registered with PROSPERO. Articles were retrieved from PubMed, EMBASE, and Scopus using relevant MeSH terms and keywords. The search, executed in October 2024, was conducted manually and with BiBot, an AI-based tool for literature reviews. Studies focusing on ML applications in osteoarthritis (OA), rheumatoid arthritis (RA), and psoriatic arthritis (PsA) were included.</div></div><div><h3>Results</h3><div>From 400 initially identified studies, 32 met the inclusion criteria. RA was the most studied disease (88 %), followed by OA (22 %) and PsA (9 %). Convolutional neural networks (CNNs) were the most frequently used algorithms (50 %). Standard radiographs (59 %) were the predominant imaging modality, followed by MRI (16 %). Despite recommendations for ML studies, external validation was conducted in only 15 % of studies, and just 6 % of datasets were publicly available. Interpretability tools were employed in 28 % of studies to enhance clinical relevance.</div></div><div><h3>Conclusion</h3><div>ML has significant potential to improve diagnostics and disease management in hand imaging of RMDs. However, key challenges remain, including the need for increased external validation, broader disease coverage (OA and PsA), and improved data-sharing practices to enhance reproducibility and clinical adoption.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152750"},"PeriodicalIF":4.6,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143931400","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}
Karin Wadström , Ola Börjesson , John Moshtaghi-Svensson , Annette Bruchfeld , Iva Gunnarsson , Marie Holmqvist
{"title":"Incidence and prevalence of granulomatosis with polyangiitis in Sweden, 2006–2019, a register-based study","authors":"Karin Wadström , Ola Börjesson , John Moshtaghi-Svensson , Annette Bruchfeld , Iva Gunnarsson , Marie Holmqvist","doi":"10.1016/j.semarthrit.2025.152745","DOIUrl":"10.1016/j.semarthrit.2025.152745","url":null,"abstract":"<div><h3>Objective</h3><div>To investigate incidence and prevalence of granulomatosis with polyangiitis (GPA) in Sweden nationwide between 1 January 2006 until 31 December 2019 in a register-based study including the entire Swedish population.</div></div><div><h3>Method</h3><div>In the population-based National Patient Register (NPR) we identified patients with incident and prevalent GPA during the period 2006–2019. Age- and sex standardized annual incidence and crude period prevalence were estimated. Results were stratified on age, sex, and season.</div></div><div><h3>Results</h3><div>We identified 2013 individuals with incident GPA during the study period. Median age was 63 years (IQR 51–72) and 46 % were women. The mean standardized incidence was 1.9 per 100,000 person-years (95 % CI 1.8–2.0), with a slightly higher incidence in men 2.0 (95 % CI 1.9–2.2) than in women 1.7 (95 % CI 1.6–1.8). We noted the highest incidence in the group aged 70–79, 4.1 (95 % 3.7–4.5). The annual incidence remained stable over the study period range 1.7–2.0. We could not find any seasonal variation in incidence. The point prevalence on December 31st, 2019, was 25.4 per 100,000 (95 % CI 24.3–26.5) based on the 2132 individuals we identified as prevalent. The period prevalence increased from 18.7 per 100,000 (95 % CI 17.8–19.1) in 2006–2010 to 23.9 per 100,000 (95 % CI 22.8–25.0) in 2016–2019.</div></div><div><h3>Conclusion</h3><div>Our estimates show that incidence of GPA has been stable over the period 2006–2019 in Sweden which are in line with published data from southern Sweden. The prevalence increased during the study period which could be due to improvement in treatment leading to an increased survival.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152745"},"PeriodicalIF":4.6,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143916933","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}
Durmalouk Kesibi, Michael Rotondi, Heather Edgell, Hala Tamim
{"title":"A cohort study on the associations between age at natural menopause and rheumatoid arthritis in postmenopausal women from the Canadian Longitudinal Study on Aging","authors":"Durmalouk Kesibi, Michael Rotondi, Heather Edgell, Hala Tamim","doi":"10.1016/j.semarthrit.2025.152747","DOIUrl":"10.1016/j.semarthrit.2025.152747","url":null,"abstract":"<div><div>Menopause represents a significant phase in a woman’s life, marked by profound physiological changes. An early onset of menopause has been associated with a variety of negative outcomes. Estrogen has been shown to be protective of bone and joint health. Hormonal links to rheumatoid arthritis have been found; previous studies exploring age at natural menopause (ANM) and Rheumatoid arthritis have produced conflicting results. This study investigated the association between ANM and incidence of rheumatoid arthritis among postmenopausal Canadian women. The study included women between the ages of 45–85 years from the Canadian Longitudinal Study on Aging followed over a 10-year period. Analysis was restricted to naturally postmenopausal women that did not have rheumatoid arthritis prior to menopause. ANM was examined using the following categories ≤ 44 (reference), 45–49, and ≥50. Survival analysis was used to determine time to onset of rheumatoid arthritis. Unadjusted and adjusted multivariable Cox regression models were used to examine the relationship between ANM and incidence of rheumatoid arthritis. The adjusted multivariable Cox regression model showed significantly lower risk of rheumatoid arthritis in women with an older ANM of ≥50 years and who have been on hormone replacement therapy for ≥8 years with a hazard ratio of 0.2 (95 % CI: 0.1–0.7) compared to women with an ANM ≤ 44 who have never used hormone replacement therapy. Our findings suggest a potential beneficial effect of longer estrogen exposure on the risk of developing rheumatoid arthritis.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152747"},"PeriodicalIF":4.6,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143924367","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}
Alessandra Ida Celia , Giovanni Maria Vescovo , Gianmarco Sarto , Cristiano Alessandri , Antonio Iaconelli , Domenico D’Amario , Giacomo Frati , Fabrizio Conti , Sebastiano Sciarretta , Dominick J Angiolillo , Andrea Fava , Michelle A Petri , Behnood Bikdeli , Mattia Galli
{"title":"Direct oral anticoagulants versus Vitamin K antagonists in antiphospholipid syndrome: A systematic review and meta-analysis","authors":"Alessandra Ida Celia , Giovanni Maria Vescovo , Gianmarco Sarto , Cristiano Alessandri , Antonio Iaconelli , Domenico D’Amario , Giacomo Frati , Fabrizio Conti , Sebastiano Sciarretta , Dominick J Angiolillo , Andrea Fava , Michelle A Petri , Behnood Bikdeli , Mattia Galli","doi":"10.1016/j.semarthrit.2025.152741","DOIUrl":"10.1016/j.semarthrit.2025.152741","url":null,"abstract":"<div><h3>Background</h3><div>Randomized controlled trials (RCTs) comparing the efficacy and safety of direct oral anticoagulants (DOACs) versus Vitamin K antagonists (VKAs) in patients with thrombotic antiphospholipid syndrome (APS) have yielded inconsistent results, partly due to the inherent challenges of conducting RCTs in populations with rare medical conditions. We conducted a systematic review and meta-analysis to evaluate the comparative effects of DOACs versus VKAs in thrombotic APS.</div></div><div><h3>Methods</h3><div>RCTs and observational studies comparing DOACs versus VKAs in patients with thrombotic APS were included. The primary endpoint was a composite of arterial (ATE) and venous thrombotic events (VTE). Incidence rate ratios (IRRs) and associated 95 % confidence intervals (CI) were used to account for different follow-up durations. GRADE was used for rating the certainty of evidence.</div></div><div><h3>Findings</h3><div>Twelve studies, four randomized and eight observational, encompassing a total of 1307 APS patients were included. The use of DOACs was associated with an increase in the primary endpoint (IRR 2.33; 95 % CI 1.18–4.58; GRADE=moderate) driven by increased ATE (IRR 2.70; 95 % CI 1.42–5.13; GRADE=low), compared with the use of VKA. VTE (IRR 0.98; 95 % CI 0.59–1.64; GRADE=low), major (IRR 0.83; 95 % CI 0.48–1.43; GRADE=low) and non-major (IRR 1.32; 95 % CI 0.81–2.14; GRADE=very low) bleeding did not differ significantly between groups. Compared with VKAs, DOACs were associated with an increase in myocardial infarction (IRR 4.71; 95 % CI 1.00–22.21; GRADE=very low) and stroke (IRR 7.48; 95 % CI 1.27–44.13; GRADE=very low). The increased risk of arterial thrombotic events with DOACs was consistently observed in a dedicated analysis of RCTs and was mitigated by the concomitant use of single antiplatelet therapy.</div></div><div><h3>Interpretation</h3><div>In patients with thrombotic APS, the use of DOACs is associated with increased thrombotic events compared with VKAs, mainly driven by arterial thrombotic events. A single antiplatelet therapy combined with DOACs maight offer a promising alternative to VKAs, warranting further dedicated investigations.</div></div><div><h3>Primary Funding Source</h3><div>The study was not funded.</div></div><div><h3>Protocol registration</h3><div>This study is registered in PROSPERO (CRD42024582033).</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152741"},"PeriodicalIF":4.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143916934","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":"Validation study of the SER/SEPAR screening criteria for interstitial lung disease in early rheumatoid arthritis patients","authors":"Martí Aguilar-Coll, Javier Narváez","doi":"10.1016/j.semarthrit.2025.152738","DOIUrl":"10.1016/j.semarthrit.2025.152738","url":null,"abstract":"<div><h3>Objective</h3><div>In 2023, the Spanish Society of Rheumatology (SER) and the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) proposed screening criteria for interstitial lung disease (ILD) in rheumatoid arthritis (RA) based on expert opinion, requiring validation. This study aimed to evaluate their sensitivity and specificity in a cohort of early RA patients.</div></div><div><h3>Methods</h3><div>This cross-sectional study retrospectively assessed the SER/SEPAR criteria in 146 early RA patients screened for ILD at diagnosis. Screening included medical history, respiratory auscultation, chest X-ray (CXR), and complete pulmonary function tests (PFTs). Thoracic high-resolution computed tomography (HRCT) was performed only in the presence of symptoms, velcro crackles, or abnormalities on CXR or PFTs.</div></div><div><h3>Results</h3><div>Among the 146 patients included, 28 (19.2 %) developed ILD, all confirmed by HRCT. Of these, 12 (43 %) had clinically evident ILD preceding or coinciding with joint symptoms, while the remaining 16 (57 %) were identified after applying the screening protocol. At diagnosis, 90 patients (61.6 %) met the screening criteria, with ILD confirmed in 26 cases (28.9 %). Conversely, among 56 patients (38.4 %) not meeting the criteria, ILD was ultimately identified in 2 cases (3.5 %). The SER/SEPAR screening criteria demonstrated a sensitivity of 92.9 % (95 % CI: 76.5–99.1), specificity of 45.8 % (95 % CI: 36.6–55.2), LR+ of 1.71 (95 % CI: 1.41–2.08), LR– of 0.16 (95 % CI: 0.04–0.60), PPV of 28.9 % (95 % CI: 25.1–33.1), NPV of 96.4 % (95 % CI: 87.5–99.1), and diagnostic accuracy of 54.8 % (95 % CI: 46.4–63.1).</div></div><div><h3>Conclusion</h3><div>The SER/SEPAR criteria for ILD screening demonstrated high sensitivity in recent-onset RA patients, supporting their utility as a tool for early detection in this scenario.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152738"},"PeriodicalIF":4.6,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143911601","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}
Jessica L Fairley , Laura Ross , Elizabeth Paratz , Penelope McKelvie , Dylan Hansen MBiostatistics , Wendy Stevens , Andre La Gerche , Mandana Nikpour
{"title":"Pathological contributors to organ damage and mortality in systemic sclerosis: a nationwide matched case-control study","authors":"Jessica L Fairley , Laura Ross , Elizabeth Paratz , Penelope McKelvie , Dylan Hansen MBiostatistics , Wendy Stevens , Andre La Gerche , Mandana Nikpour","doi":"10.1016/j.semarthrit.2025.152739","DOIUrl":"10.1016/j.semarthrit.2025.152739","url":null,"abstract":"<div><h3>Background</h3><div>Mortality is increased in systemic sclerosis (SSc), including a 10–15-fold increase in sudden cardiac death. Limited histopathology data exist to understand the pathogenesis of this excess. This study aimed to compare heart, lung and renal histopathology at autopsy between SSc and controls.</div></div><div><h3>Methods</h3><div>A matched case-control study was performed using autopsy data from the Australian National Coronial Information System. SSc cases were selected via keyword search, and age- and sex-matched to controls identified from motor vehicle accidents. Cause of death, antemortem comorbidities and autopsy findings were extracted. Odds ratios were calculated with 95 % confidence intervals (CI).</div></div><div><h3>Findings</h3><div>Fifty-nine SSc cases (64 years, 81 % female) were matched to 59 controls (62 years, 81 % female). Myocardial fibrosis was 11-times more common in SSc (95 %CI 4–29, <em>p</em> < 0.01), inflammation 39-times more common (95 %CI 2–672, <em>p</em> = 0.01) and small vessel vasculopathy 27-times more common (95 %CI 2–485, <em>p</em> = 0.02), despite no difference in epicardial coronary artery disease (<em>p</em> = 0.24). Two-thirds of SSc cases had myocardial fibrosis with no identifiable secondary cause (e.g., coronary/valvular lesions). Pulmonary fibrosis, inflammation and vasculopathy ranged from 23 to 100-times more common in SSc. Renal fibrosis/scarring, inflammation/infiltrates and vasculopathy were 3–6-times more common in SSc. Among SSc cases, combined pathologies (≥2 of fibrosis, inflammation or vasculopathy) were seen concurrently in 31 % of hearts, 41 % of lungs and 45 % of kidneys.</div></div><div><h3>Interpretation</h3><div>The high frequency of unexplained myocardial fibrosis in SSc provides insights into the mechanism of excess mortality and sudden cardiac death observed in SSc. This matched autopsy study demonstrates the mechanisms and complexity of organ damage in SSc, with 30–45 % of organs displaying multiple concurrent pathologies.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152739"},"PeriodicalIF":4.6,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143924196","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}