RheumatologyPub Date : 2025-06-13DOI: 10.1093/rheumatology/keaf340
Veysel Cam, Dilara Unal, Erdal Sag, Yagmur Bayindir, Emil Aliyev, Hulya Ercan Emreol, Mehmet Orhan Erkan, Ozlem Necipoglu Banak, Hazel Delal Dara Kar, Ozge Basaran, Yelda Bilginer, Seza Ozen
{"title":"Re-evaluation of MEFV carriers previously diagnosed with familial Mediterranean fever: a colchicine discontinuation study.","authors":"Veysel Cam, Dilara Unal, Erdal Sag, Yagmur Bayindir, Emil Aliyev, Hulya Ercan Emreol, Mehmet Orhan Erkan, Ozlem Necipoglu Banak, Hazel Delal Dara Kar, Ozge Basaran, Yelda Bilginer, Seza Ozen","doi":"10.1093/rheumatology/keaf340","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf340","url":null,"abstract":"<p><strong>Objectives: </strong>Familial Mediterranean Fever (FMF) is an autoinflammatory disease associated with mutations in the MEFV gene. While typically inherited in an autosomal recessive pattern, heterozygous individuals may also exhibit FMF symptoms, often with a milder disease course. The long-term management of colchicine therapy in heterozygous patients, particularly decisions regarding its discontinuation, remains a clinical challenge.</p><p><strong>Methods: </strong>This retrospective cohort study evaluated pediatric patients with a heterozygous pathogenic MEFV mutation who were followed at a single tertiary center between September 2024 and March 2025. Both patients in whom colchicine therapy was successfully discontinued and those in whom discontinuation was not feasible were analyzed. Clinical characteristics, attack features, inflammatory markers, and treatment outcomes were assessed. Multivariate logistic regression and ROC curve analyses were performed to identify predictors of successful colchicine discontinuation.</p><p><strong>Results: </strong>A total of 136 patients were included. Of the 84 patients who attempted colchicine discontinuation, 72 (85.7%) remained off therapy, while 12 (14.3%) resumed treatment. Early absence of attacks during follow-up was associated with successful colchicine discontinuation., whereas arthritis predicted continued treatment. ROC analysis showed that a ≥ 70.8% reduction in attack frequency during the first six months of therapy strongly predicted successful discontinuation (AUC = 0.883, 95% CI: 0.823-0.943).</p><p><strong>Conclusion: </strong>Our findings suggest that colchicine therapy can be safely discontinued in selected heterozygous individuals who show early absence of attacks, suggesting that the initial diagnosis of FMF in some patients may warrant reconsideration. However, it is important to closely monitor these children after treatment cessation, and decisions should be guided by careful follow-up and regular reassessment.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144286406","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":"Bidirectional association of rheumatoid arthritis and inflammatory bowel diseases: a large-scale prospective cohort study.","authors":"Yijun Chen, Qian Zhang, Si Liu, Shengtao Zhu, Jing Wu, Shutian Zhang, Peng Li, Shanshan Wu","doi":"10.1093/rheumatology/keaf337","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf337","url":null,"abstract":"<p><strong>Objectives: </strong>to investigate the bidirectional prospective association between inflammatory bowel disease (IBD) and rheumatoid arthritis (RA) in a large-scale, long-term follow-up, population-based cohort.</p><p><strong>Methods: </strong>Participants free of any cancer at baseline were included and divided into two prospective cohorts: baseline IBD and incident RA cohort (cohort 1), and baseline RA and incident IBD cohort (cohort 2), respectively. The primary outcome was incident RA in cohort 1 and incident IBD, including ulcerative colitis (UC) and Crohn's disease (CD) in cohort 2, separately. Cox proportional hazard regression models were used to investigate the bidirectional relationship between RA and IBD.</p><p><strong>Results: </strong>Overall, 449 662 and 450 534 participants were included in cohort 1 and cohort 2, with 5,015/5,887 prevalent IBD/RA cases at baseline, respectively. During a median of 14.3/14.6-year follow-up, 6,001(1.3%)/2,988(0.7%) cases of RA and IBD were identified in each cohort. Compared with non-IBD, IBD patients (HR = 1.44, 95% CI: 1.15-1.79) showed a significantly higher risk of incident RA, particularly in UC patients (HR = 1.36, 95% CI: 1.06-1.75) after multivariable adjustment. Similarly, RA patients had a 1.65-fold higher risk (95% CI: 1.31-2.09) of incident IBD, with a 60% and 65% excess risk of developing UC (HR = 1.60, 95% CI: 1.20-2. 13) and CD (HR = 1.65, 95% CI: 1.12-2. 42), respectively. Further sensitivity analysis and subgroup analysis indicated similar results.</p><p><strong>Conclusion: </strong>IBD is associated with an increased risk of RA, and vice versa. Further studies are warranted to confirm the findings and elucidate the underlying biological mechanisms.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144286405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2025-06-12DOI: 10.1093/rheumatology/keaf240
Kathryn Biddle, Judith Jade, Harold Wilson-Morkeh, Madura Adikari, Chadwan Al Yaghchi, Zoi Anastasa, Neil Basu, Paul Brogan, Dimitrios Chanouzas, Shouvik Dass, David D'Cruz, Marcos Martinez Del Pero, Emmandeep Dhillon, Georgina Ducker, Siân Griffin, Rosemary J Hollick, David Jackson, Catherine King, Matko Marlais, Alice Mason, Stephen McAdoo, Devesh Mewar, Janice Mooney, Eleana Ntatsaki, Fiona Pearce, Benjamin Rhodes, Hitasha Rupani, Alan Salama, Salman Siddiqui, Rona Smith, Lorraine Harper
{"title":"The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis.","authors":"Kathryn Biddle, Judith Jade, Harold Wilson-Morkeh, Madura Adikari, Chadwan Al Yaghchi, Zoi Anastasa, Neil Basu, Paul Brogan, Dimitrios Chanouzas, Shouvik Dass, David D'Cruz, Marcos Martinez Del Pero, Emmandeep Dhillon, Georgina Ducker, Siân Griffin, Rosemary J Hollick, David Jackson, Catherine King, Matko Marlais, Alice Mason, Stephen McAdoo, Devesh Mewar, Janice Mooney, Eleana Ntatsaki, Fiona Pearce, Benjamin Rhodes, Hitasha Rupani, Alan Salama, Salman Siddiqui, Rona Smith, Lorraine Harper","doi":"10.1093/rheumatology/keaf240","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf240","url":null,"abstract":"<p><p>ANCA-associated vasculitis (AAV) is comprised of three specific conditions: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Since the publication of the last British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guideline for the management of adults with AAV in 2014, a plethora of randomized controlled trials, additional research and recommendations have provided novel insights into how the management of AAV can be optimized, thus improving patient quality of life. The BSR AAV Working Group (WG) reviewed published guidelines, undertook a systematic literature review and utilized expertise from specialist vasculitis centres across the UK and patient representatives to formulate a list of 26 recommendations with corresponding strength of agreement (SOA) scores. Recommendations were updated from the published 2014 BSR and BHPR guideline. The 26 recommendations encompassed five key domains: 1. Treatment for GPA and MPA; 2. Management of subglottic stenosis and ear, nose and throat (ENT) manifestations of AAV; 3. Management and treatment for EGPA; 4. Service specifications; 5. Patient education and support. These recommendations provide an update on care delivery of AAV based on current evidence and specialist opinion. In addition, we have provided research and audit recommendations to support equitable access to care and improve health outcomes. The lay summary that accompanies this abstract can be found in Supplementary Data S1, available at Rheumatology online.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2025-06-12DOI: 10.1093/rheumatology/keaf329
Saverio La Bella, Deniz Bayraktar, Annamaria Porreca, Linda C Li, Marina Attanasi, Emil Aliyev, Angela Nyangore Migowa, Christiaan Scott, Darpan R Thakare, Yagmur Bayindir, Alessandro Consolaro, Brian M Feldman, Seza Ozen
{"title":"Global variations in artificial intelligence-generated information on juvenile idiopathic arthritis.","authors":"Saverio La Bella, Deniz Bayraktar, Annamaria Porreca, Linda C Li, Marina Attanasi, Emil Aliyev, Angela Nyangore Migowa, Christiaan Scott, Darpan R Thakare, Yagmur Bayindir, Alessandro Consolaro, Brian M Feldman, Seza Ozen","doi":"10.1093/rheumatology/keaf329","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf329","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to evaluate similarities and variations of information provided by Large Language Models (LLMs) across diverse world regions by analyzing responses to validated questions on oligoarticular juvenile idiopathic arthritis (oJIA).</p><p><strong>Methods: </strong>The ten PICOs related to the oJIA treatment on the 2021 American College of Rheumatology recommendations were simultaneously prompted in English to ChatGPT 4o from five different countries (Canada, India, Italy, Kenya and Türkiye). Readability was assessed through the Flesch Reading Ease Score (FRES), distinctiveness of terms through the Term Frequency-Inverse Document Frequency (TF-IDF) analysis. Co-occurrence networks (CONs) detailed the relationships between terms. Three experts rated the adherence of responses to recommendations using a Likert-like scale.</p><p><strong>Results: </strong>All the responses were difficult or very difficult to read, with a median FRES of 30 [24-34]. Depending on the expert, 52% to 84% of responses were mostly or fully adherent to the recommendations, with similar adherence rates across countries. No response was not adherent at all. Inter-rater agreement on the adherence of LLM-generated responses was generally weak (Kappa values mostly below 0.40), highlighting the challenges of consistently evaluating AI-generated medical information. The TF-IDF analysis showed that the distinctiveness of terminology in LLM-generated responses varied across countries, with scores ranging from 0.60-0.85. CONs detailed a strong focus on intra-articular corticosteroid treatments in Italy and emphasis on short- and long-term outcomes in Kenya.</p><p><strong>Conclusion: </strong>LLM-generated content should be critically evaluated in clinical practice, especially in the context of regional differences.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275762","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2025-06-12DOI: 10.1093/rheumatology/keaf332
Matteo Piga
{"title":"Lupus serology: off target but still relevant to the treat-to-target strategy in systemic lupus erythematosus.","authors":"Matteo Piga","doi":"10.1093/rheumatology/keaf332","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf332","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2025-06-12DOI: 10.1093/rheumatology/keaf328
Davide Mohammad Reza Beigi, Greta Pellegrino, Nicholas Landini, Marco Emanuele Diana, Gregorino Paone, Ilaria Bisconti, Francesca Romana Di Ciommo, Marius Cadar, Elena Platania, Jacopo Landro, Simona Truglia, Valeria Panebianco, Fabrizio Conti, Valeria Riccieri
{"title":"Comparison of quantitative lung ultrasound scores with automated quantitative CT: to overcome the ultrasound limitations.","authors":"Davide Mohammad Reza Beigi, Greta Pellegrino, Nicholas Landini, Marco Emanuele Diana, Gregorino Paone, Ilaria Bisconti, Francesca Romana Di Ciommo, Marius Cadar, Elena Platania, Jacopo Landro, Simona Truglia, Valeria Panebianco, Fabrizio Conti, Valeria Riccieri","doi":"10.1093/rheumatology/keaf328","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf328","url":null,"abstract":"<p><strong>Objectives: </strong>Lung ultrasound (LUS) is emerging as a valuable tool for assessing systemic sclerosis-associated interstitial lung disease (SSc-ILD), although it traditionally explores only superficial lung regions. Building upon our preliminary findings, this study investigated correlations between quantitative LUS scores and automated quantitative computed tomography (qCT) measurement of ILD extent, including both superficial and deeper lung involvement.</p><p><strong>Methods: </strong>Between 2021 and 2023, 82 consecutive SSc patients underwent concurrent LUS and CT scans. Total B-lines (BL) count (range 0-140) and our novel pleural line irregularity (PLI) score (range 0-28) were obtained using a 14-intercostal space scanning protocol. CT scans were analysed by automated texture analysis software, quantifying volumes of ILD, ground-glass opacities (GGO) and reticulations (RET), segmented in three levels (apices, midfields, bases) and subdivided in surface and core lung parenchyma.</p><p><strong>Results: </strong>Total BL count and PLI score correlated with total ILD, GGO and RET volumes (all p< 0.0001), as well as with surface and core ILD volumes (all p< 0.0001). Basal lung BLs and PLI score correlated with basal ILD, GGO, RET (all p< 0.005), and corresponding surface and core ILD volumes (all p< 0.005). Mid-lung PLI correlated also with corresponding ILD-related changes and surface and core ILD (all p< 0.005). These associations were confirmed by multivariate regression analysis.</p><p><strong>Conclusions: </strong>Quantitative LUS score correlated with qCT-defined ILD extent, especially at lung bases. LUS scores (particularly the novel PLI score) were found to correlate with deeper ILD volume, suggesting potential to overcome traditional LUS limitations related to superficial lung assessment.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275760","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2025-06-12DOI: 10.1093/rheumatology/keaf242
Kathryn Biddle, Judith Jade, Harold Wilson-Morkeh, Madura Adikari, Chadwan Al Yaghchi, Zoi Anastasa, Neil Basu, Paul Brogan, Dimitrios Chanouzas, Shouvik Dass, David D'Cruz, Marcos Martinez Del Pero, Emmandeep Dhillon, Georgina Ducker, Siân Griffin, Rosemary J Hollick, David Jackson, Catherine King, Matko Marlais, Alice Mason, Stephen McAdoo, Devesh Mewar, Janice Mooney, Eleana Ntatsaki, Fiona Pearce, Benjamin Rhodes, Hitasha Rupani, Alan Salama, Salman Siddiqui, Rona Smith, Lorraine Harper
{"title":"Executive summary: The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis.","authors":"Kathryn Biddle, Judith Jade, Harold Wilson-Morkeh, Madura Adikari, Chadwan Al Yaghchi, Zoi Anastasa, Neil Basu, Paul Brogan, Dimitrios Chanouzas, Shouvik Dass, David D'Cruz, Marcos Martinez Del Pero, Emmandeep Dhillon, Georgina Ducker, Siân Griffin, Rosemary J Hollick, David Jackson, Catherine King, Matko Marlais, Alice Mason, Stephen McAdoo, Devesh Mewar, Janice Mooney, Eleana Ntatsaki, Fiona Pearce, Benjamin Rhodes, Hitasha Rupani, Alan Salama, Salman Siddiqui, Rona Smith, Lorraine Harper","doi":"10.1093/rheumatology/keaf242","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf242","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275761","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2025-06-10DOI: 10.1093/rheumatology/keaf314
Ana Serrano-Combarro, Belén Atienza-Mateo, Adrián Martín-Gutiérrez, Jesús Loarce Martos, César Antonio Egües Dubuc, Marta Pastor Mena, Rafael B Melero-Gonzalez, María Martín López, Natalia Mena Vázquez, Carmen Carrasco-Cubero, Carolina Pérez García, Andrea García Valle, Gema Bonilla, Juan María Blanco Madrigal, Uxue Astigarraga-Urquia, Nuria Vegas Revenga, Lorena Pérez Albadalejo, Rafaela Ortega Castro, Deseada Palma Sánchez, Ana María Fernández Ortiz, Patricia López Viejo, María López Lasanta, Marta Garijo Bufort, Ivette Casafont Solé, José Ramón Lamua-Riazuelo, Ignacio Braña Abascal, Virginia Ruiz-Esquide, Evelin Cervantes Perez, Bryan-Josué Flores Robles, María Paz Martínez-Vidal, Juan Moreno Morales, Ana Urruticoechea-Arana, José Rosas, Delia Fernández Lozano, David Castro Corredor, Iván Ferraz-Amaro, Santos Castañeda, Ricardo Blanco
{"title":"Baricitinib in rheumatoid arthritis-interstitial lung disease: national multicenter study of 72 patients and literature review.","authors":"Ana Serrano-Combarro, Belén Atienza-Mateo, Adrián Martín-Gutiérrez, Jesús Loarce Martos, César Antonio Egües Dubuc, Marta Pastor Mena, Rafael B Melero-Gonzalez, María Martín López, Natalia Mena Vázquez, Carmen Carrasco-Cubero, Carolina Pérez García, Andrea García Valle, Gema Bonilla, Juan María Blanco Madrigal, Uxue Astigarraga-Urquia, Nuria Vegas Revenga, Lorena Pérez Albadalejo, Rafaela Ortega Castro, Deseada Palma Sánchez, Ana María Fernández Ortiz, Patricia López Viejo, María López Lasanta, Marta Garijo Bufort, Ivette Casafont Solé, José Ramón Lamua-Riazuelo, Ignacio Braña Abascal, Virginia Ruiz-Esquide, Evelin Cervantes Perez, Bryan-Josué Flores Robles, María Paz Martínez-Vidal, Juan Moreno Morales, Ana Urruticoechea-Arana, José Rosas, Delia Fernández Lozano, David Castro Corredor, Iván Ferraz-Amaro, Santos Castañeda, Ricardo Blanco","doi":"10.1093/rheumatology/keaf314","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf314","url":null,"abstract":"<p><strong>Objective: </strong>To assess the effectiveness and safety of baricitinib (BARI) in Rheumatoid Arthritis-interstitial lung disease (RA-ILD) in clinical practice.</p><p><strong>Methods: </strong>: National multicentre retrospective study of 72 RA-ILD patients treated with BARI. We analyzed the following outcomes at baseline and at 3, 6, 12, 18, 24 months, and last follow-up: a) dyspnea (modified Medical Research Council scale), b) forced vital capacity (FVC), c) diffusing capacity of the lungs for carbon monoxide (DLCO), d) chest high resolution computed tomography (HRCT), e) arthritis activity (DAS28-ESR), and f) corticosteroid-sparing effect. Additionally, we analyzed safety data and performed a literature review up to now.</p><p><strong>Results: </strong>We included 72 patients (52 women; mean age 68 ± 10 years). All patients had received disease-modifying antirheumatic drugs (DMARDs). Median ILD duration up to BARI initiation was of 25 [13-63] months. Most frequent ILD patterns were usual interstitial pneumonia (n = 33; 49%) and non-specific interstitial pneumonia (n = 22; 32%). BARI was used in monotherapy in 43 (60%) patients and combined with conventional DMARDs in 29 (40%). Mean baseline values of FVC and DLCO (% pred.) were 86 ± 28 and 69 ± 20, respectively. After a median [IQR] follow-up of 32 [13-65] months dyspnea, FVC, DLCO, HRCT improved/stabilized in 90%, 88%, 65%, 72%, respectively. Mean DAS28-ESR improved from 4.29-2.99. and median prednisone dose was reduced from 5 to 2.5 mg/day. Relevant adverse events were uncommon.</p><p><strong>Conclusion: </strong>BARI may be a useful and safe alternative in both pulmonary and joint disease in RA-ILD patients, even in refractory cases.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144267149","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2025-06-10DOI: 10.1093/rheumatology/keaf324
Claire Poggi, Eric Hachulla, Alexandre Karras, Antoine Briantais, Camille Ravaiau, Pierre Gobert, Alban Deroux, Sarah Nicolas, Nabil Belfeki, Hélène François, Matthieu Groh, Jonathan London, Julien Campagne, Jean-Sébastien Allain, Emmanuelle Dernis, Cécile-Audrey Durel, Thomas Le Gallou, Alexandre Curie, Philippe Kerschen, Noémie Gensous, Anne-Hélène Reboux, Hélène Béhal, Benjamin Terrier, Thomas Quéméneur
{"title":"Prognosis of essential mixed cryoglobulinemia and connective tissue disease-related cryoglobulinemia after rituximab-induced remission","authors":"Claire Poggi, Eric Hachulla, Alexandre Karras, Antoine Briantais, Camille Ravaiau, Pierre Gobert, Alban Deroux, Sarah Nicolas, Nabil Belfeki, Hélène François, Matthieu Groh, Jonathan London, Julien Campagne, Jean-Sébastien Allain, Emmanuelle Dernis, Cécile-Audrey Durel, Thomas Le Gallou, Alexandre Curie, Philippe Kerschen, Noémie Gensous, Anne-Hélène Reboux, Hélène Béhal, Benjamin Terrier, Thomas Quéméneur","doi":"10.1093/rheumatology/keaf324","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf324","url":null,"abstract":"Objectives Rituximab (RTX) and glucocorticoids are the first line treatment for essential (EM) and connective tissue disease (CTD)-related mixed cryoglobulinemia vasculitis (CryoVas). Data on long term outcomes of these CryoVas are lacking. We aimed to describe the prognosis of patients with EM and CTD-related CryoVas. Methods We conducted a retrospective study on patients with EM or CTD-related CryoVas in remission after RTX-based therapy. Results We included 63 patients with a median follow-up of 58 months (IQR, 33–88 months). Relapse rates were 23% at 1 year, 42% at 2 years and 71% at 5 years after the initial flare. In univariate analysis, factors associated with relapse were purpura (HR, 2.2; 95% confidence interval (CI), 1.1–4.4; p = 0.02) and a previous flare of CryoVas (HR, 1.9; 95% CI, 1.0–3.7; p = 0.04). Maintenance therapy was associated with a lower risk of early relapse (HR, 0.3; 95% CI, 0.1–0.9; p = 0.03), but not of late relapse (HR, 2.0; 95% CI, 0.7–5.7; p = 0.21). In multivariable analysis, patients without purpura or previous flare remained at lower risk of relapse than those with at least one of the two (HR, 3.6; 95%CI, 1.6–8.2; p= 0.002). Maintenance regimen was associated with a lower risk of early relapse (HR, 0.3; 95% CI, 0.1–0.9; p = 0.03). Conclusion In patients with EM and CTD-related CryoVas who received RTX as induction therapy, relapses were frequent and associated with purpura and a previous flare, but were reduced with maintenance therapy.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"145 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144259970","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}