Rheumatology最新文献

{"title":"Temporal trends in vascular medication use in 8,079 patients with systemic sclerosis: insights to inform future trials and therapeutic strategies from the EUSTAR cohort.","authors":"Stefano Di Donato, John D Pauling, Sheila Ramjug, Yannick Allanore, Edward B Jude, Marie-Elise Truchetet, Paolo Airò, Lidia P Ananyeva, Andra Balanescu, Gonçalo Boleto, Francesco Paolo Cantatore, Patricia E Carreira, Carolina de Souza Müller, Masataka Kuwana, Gianluca Moroncini, Marco Di Battista, Luc Mouthon, Madelon C Vonk, Elisabetta Zanatta, Marco- Matucci-Cerinic, Francesco Del Galdo, Michael Hughes","doi":"10.1093/rheumatology/keaf290","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf290","url":null,"abstract":"<p><strong>Objectives: </strong>Systemic sclerosis (SSc) is characterised by widespread vascular damage resulting in digital and systemic vasculopathic sequelae. Although there are effective treatments available, vascular disease remains a significant cause of morbidity and mortality in SSc. Our aim was to describe patterns of vascular medication use in SSc, including examination for potential changes over time.</p><p><strong>Methods: </strong>A cross-sectional study of SSc patients enrolled in the EUSTAR database meeting 2013 ACR/EULAR SSc criteria. Patients were divided into two time periods: 2012-2017 and 2018-2022. We analysed the prescription patterns of endothelin receptor antagonists (ERA), phosphodiesterase type-5 inhibitors (PDE5i), calcium channel blockers (CCB), intravenous iloprost, and antiplatelet therapies. Logistic regression was used to evaluate temporal trends and interaction effects.</p><p><strong>Results: </strong>8079 patients were included. Significant increases over time were observed in the use of ERA (7% to 12%, p< 0.001), PDE5i (5.4% to 7.2%, p= 0.064), CCB (20% to 32%, p< 0.001), and anti-platelet therapies (15% to 20%, p< 0.001). There was a notable decrease in iloprost use (3.1% to 0.3%, p< 0.001). The prevalence of active digital ulcers (DU) decreased (16% to 13%, p= 0.040), while a history of DU (24% to 30%, p< 0.001) increased. Year-by-year and nonlinear increases were noted for ERA and CCB whereas nonlinear increase was observed for PDE5i. Year-by-year and nonlinear decrease was observed for Iloprost prescription.</p><p><strong>Conclusion: </strong>A significant change has occurred over time in vascular medication use in SSc patients, with increased utilisation of ERA, PDE5i, CCB, and anti-platelet therapies, suggesting the adoption of more proactive and/or preventive treatment strategies.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209401","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":"JAK inhibitors alleviate metabolic dysregulation, inflammation and fibrosis in osteoarthritis: insights from human joint cells and synovium.","authors":"Geneviève Paulissen, Olivier Malaise, Céline Deroyer, Edith Charlier, Sophie Neuville, Zelda Plener, Thierry Thirion, Christophe Daniel, Clio Ribbens, Dominique de Seny","doi":"10.1093/rheumatology/keaf298","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf298","url":null,"abstract":"<p><strong>Objectives: </strong>Osteoarthritis (OA) presents a significant clinical challenge due to its heterogeneous nature, characterized by cartilage degradation, inflammation, and fibrosis. Current treatments offer limited efficacy, highlighting the need for novel therapeutic approaches. Our study aimed to investigate the effects of two JAK inhibitors, tofacitinib and baricitinib, on various hallmarks of OA in human joint cells and synovium.</p><p><strong>Methods: </strong>Human OA fibroblast-like synoviocytes (FLS), OA chondrocytes, and synovial explants were cultured with tofacitinib or baricitinib, with or without additional stimulation (IL-1β or TGF-β). The levels of p-STAT1, p-STAT3, SOX9, and α-SMA were assessed by Western blot whereas SOX9, COL2A1, ACAN, ACTA2, CTGF and COL3A1 gene expression was examined by RT-qPCR. Secreted IL-6, MMP-1, MMP-3, MMP-13 were measured in supernatants by ELISA.</p><p><strong>Results: </strong>Tofacitinib or baricitinib increased the expression of anabolic factors SOX9, COL2A1, and ACAN while decreasing MMP-13 and MMP-3 levels in OA chondrocytes. Secreted levels of IL-6 and MMP-1 were significantly reduced in IL-1β-stimulated OA FLS and in OA synovial explants treated with tofacitinib or baricitinib. Finally, baricitinib decreased some fibrotic markers: α-SMA expression, ACTA2 gene expression, and CTGF levels in TGF-β-stimulated OA FLS.</p><p><strong>Conclusion: </strong>Tofacitinib and baricitinib modulate some features of OA pathophysiology by promoting anabolic processes in OA cartilage, reducing inflammation in OA synovium, and attenuating some fibrotic factors in OA FLS. These findings demonstrate the potential use of tofacitinib and baricitinib as therapeutic options for managing OA, and highlight pathogenic pathways to target for further research and development of new OA treatment strategies.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209394","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":"Switching to IL-6 inhibitors or abatacept in elderly patients with rheumatoid arthritis achieving low disease activity with jak inhibitors: a pilot study.","authors":"Shunichi Fujita, Yoshitaka Morita, Kazuhisa Nakano","doi":"10.1093/rheumatology/keaf300","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf300","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199951","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":"Lung transplantation outcomes of patients with interstitial pneumonia with autoimmune features: a single center retrospective cohort study.","authors":"Alec Yu, Hyein Kim, Robert D Levy, Jennifer M Wilson, Darya S Jalaledin, James Choi, John Yee, Charles D Poirier, Sabrina Anh-Tu Hoa, Océane Landon-Cardinal, Kun Huang","doi":"10.1093/rheumatology/keaf299","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf299","url":null,"abstract":"<p><strong>Objective: </strong>Interstitial pneumonia with autoimmune features (IPAF) describes patients with interstitial lung disease (ILD) and autoimmune features without meeting criteria for a specific rheumatic disease. No longitudinal data exist on post-transplant outcomes in IPAF patients. We compared baseline demographics, pre-transplant characteristics, and post-transplant outcomes between IPAF and idiopathic pulmonary fibrosis (IPF) patients undergoing double lung transplantation.</p><p><strong>Methods: </strong>We retrospectively analyzed lung transplant recipients with ILD in British Columbia between Jan 1, 2014, and Apr 30, 2024. Diagnoses of IPAF and IPF were made by multidisciplinary review. Continuous variables were analyzed using the Mann-Whitney U test, categorical variables with Fisher's Exact test, and survival using Kaplan-Meier analysis.</p><p><strong>Results: </strong>We identified 20 IPAF and 64 IPF patients. IPAF patients were more likely female (50% vs 17%, p = 0.006), on pre-transplant immunosuppression (60% vs 6.3%, p < 0.001), and were less likely to receive antifibrotics (20% vs 64%, p < 0.001). No difference was seen in 1-year or cumulative survival, though survival curves diverged over time favouring IPAF. Post-transplant lung function, acute rejection, infection-related hospitalization, malignancy, and chronic lung allograft dysfunction (CLAD) were similar, with non-usual interstitial pneumonia (UIP) IPAF exhibiting a survival advantage over IPF (100% vs 66%, p = 0.044). Explant pathology revealed more UIP patterns in IPF, while IPAF showed nonspecific interstitial pneumonia (NSIP) or unclassifiable patterns.</p><p><strong>Conclusions: </strong>Post-transplant survival, lung function, and complication rates were comparable between IPAF and IPF patients at one year and the last follow-up. This is the first study to report both short- and long-term lung transplant outcomes in IPAF patients.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199950","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":"Executive Summary: The 2025 British Society for Rheumatology guideline for the treatment of axial spondyloarthritis with biologic and targeted synthetic DMARDs.","authors":"Sizheng Steven Zhao, Stephanie R Harrison, Ben Thompson, Max Yates, Joe Eddison, Antoni Chan, Nick Clarke, Nadia Corp, Charlotte Davis, Lambert Felix, Kalveer Flora, William J Gregory, Gareth T Jones, Christopher A Lamb, Helena Marzo-Ortega, Daniel J Murphy, Harry Petrushkin, Virinderjit Sandhu, Raj Sengupta, Stefan Siebert, Danielle A Van Der Windt, Dale Webb, Zenas Z N Yiu, Karl Gaffney","doi":"10.1093/rheumatology/keaf090","DOIUrl":"10.1093/rheumatology/keaf090","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"3234-3241"},"PeriodicalIF":4.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107059/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811862","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":"Efficacy of IL-23 inhibitors in axial involvements of chronic non-bacterial osteitis with palmoplantar pustulosis: a case series.","authors":"Hirotaka Yamamoto, Yoshinori Taniguchi, Shigeyoshi Tsuji, Philip S Helliwell, Mitsumasa Kishimoto","doi":"10.1093/rheumatology/keaf088","DOIUrl":"10.1093/rheumatology/keaf088","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"4081-4083"},"PeriodicalIF":4.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107040/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399890","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":"Corticosteroid use in PFAPA syndrome: clinical practice data from the JIR-CliPS Survey Study and a comprehensive literature review.","authors":"Ezgi Deniz Batu, Seher Sener, Mariana Rodrigues, Caroline Vinit, Francois Hofer, Katerina Laskari, Ricardo Craveiro Costa, Margarida Santos Faria, Gulcan Ozomay Baykal, Oksana Boyarchuk, Olivier Gilliaux, Konstantinos Pateras, Hafize Emine Sonmez, Natasa Toplak, Marco Gattorno, Michaël Hofer","doi":"10.1093/rheumatology/keaf036","DOIUrl":"10.1093/rheumatology/keaf036","url":null,"abstract":"<p><strong>Objectives: </strong>CS are used to abort disease flares in periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. We aimed to obtain a global overview of physicians' CS usage strategies and analyse the data in the literature regarding CS use in PFAPA syndrome.</p><p><strong>Methods: </strong>The Juvenile Inflammatory Rheumatism Clinical Practice Strategies (JIR-CliPS) PFAPA questionnaire included nine questions on CS use in addition to the demographic data questions. The survey was distributed via e-mail to potential respondents. The MEDLINE/PubMed and Scopus databases were searched systematically to extract the data regarding CS use in PFAPA syndrome.</p><p><strong>Results: </strong>From 47 countries, 144 physicians (female/male = 2.6; 67.4% paediatric rheumatologists) answered the survey. Most respondents (n = 133; 92.4%) prescribe CS in PFAPA flares. The most frequently prescribed CS was prednisolone (63.2%). The definition of response to CS was indicated as 'response within 12 h' by the highest number of respondents (n = 61; 42.4%). When CS cause an increase in attack frequency, most (57.9%) consider another treatment if this causes a decrease in quality of life. Forty-four (30.6%) respondents were 'routinely' prescribing CS to PFAPA patients, and this practice was more frequent among more experienced physicians (P < 0.001). We identified 46 articles in the literature describing 4564 PFAPA patients treated with CS. Prednisone was the most frequently preferred CS (48.2%). Response to CS was around 95%, although an increase in attack frequency was noted in almost 35% of the patients.</p><p><strong>Conclusion: </strong>Physicians frequently use CS for PFAPA in their routine clinical practice. Regarding treatment modification, the quality of life was a prominent consideration for physicians.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"3787-3796"},"PeriodicalIF":4.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383027","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":"Cerebrovascular accidents in giant cell arteritis: prevalence and predictive factors from the ARTESER registry.","authors":"Adrián Martín-Gutiérrez, Juan Molina-Collada, Marta Domínguez-Álvaro, Rafael B Melero-González, Elisa Fernández-Fernández, Maite Silva-Díaz, Jesús Alejandro Valero, Ismael González, Julio Sánchez Martín, Javier Narváez, Itziar Calvo, Vicente Aldasoro, Lydia Abasolo Alcázar, Javier Loricera, Alberto Ruíz-Roman, Santos Castañeda, Clara Molina-Almela, María Alcalde Villar, Antonio Juan Mas, Ricardo Blanco","doi":"10.1093/rheumatology/keaf051","DOIUrl":"10.1093/rheumatology/keaf051","url":null,"abstract":"<p><strong>Objective: </strong>To determine the prevalence and predictive factors of cerebrovascular accidents (CVA) in GCA.</p><p><strong>Methods: </strong>ARTESER is a large Spanish multicentre registry including patients with GCA from across the entire country diagnosed between June 2013 and March 2019 and sponsored by the Spanish Society of Rheumatology. The variables collected at diagnosis were demographics, clinical manifestations (including the occurrence and location of CVA), laboratory, histology and imaging findings. Patients with and without CVA were compared in a bivariate analysis. Multivariate logistic regression was performed to determine potential predictive factors of CVA.</p><p><strong>Results: </strong>A total of 1540 patients with GCA were included for analysis (mean age 77.1 years, 70% females). CVA occurred in 61 (3.96%), of whom 38 (62.3%) involved the vertebrobasilar territory and 21 (34.4%) the carotid territory. The factors associated with CVA were the occurrence of transient ischaemic attack (TIA) [odds ratio (OR) 8.63; 95% CI 2.877-25.86], large vessel (LV) involvement (OR 2.79; 95% CI 1.421-5.465) and the presence of concomitant visual manifestations (OR 2.73; 95% CI 1.427-5.235). The risk of death during follow-up was significantly higher in patients with CVA (18% vs 8.8%; P = 0.014). Patients with CVA received significantly higher mean prednisone (mg) dose at diagnosis (433.9 vs 216; P < 0.001) and cumulative prednisone dose during follow-up (11 203.9 vs 8,194.1; P < 0.001).</p><p><strong>Conclusion: </strong>The prevalence of CVA in patients with GCA is low, but increases the risk of mortality. The presence of TIA, LV involvement and visual manifestations are factors associated with increased risk of CVA.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"3733-3738"},"PeriodicalIF":4.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143371181","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":"Efficacy and safety of avacopan in patients aged 65 years and older with ANCA-associated vasculitis: a post hoc analysis of data from the ADVOCATE trial.","authors":"Duvuru Geetha, Christian Pagnoux, Sebastian E Sattui, Peter A Merkel, Maria Weiner, Juliana Draibe, Stanislas Faguer, Sarah Bray, Rachel E Gurlin, Monica Balcells-Oliver, Annette Bruchfeld, David R Jayne","doi":"10.1093/rheumatology/keaf122","DOIUrl":"10.1093/rheumatology/keaf122","url":null,"abstract":"<p><strong>Objectives: </strong>To evaluate the efficacy and safety of avacopan in patients aged ≥65 years with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) in the phase 3 ADVOCATE trial of avacopan vs a prednisone taper, plus either rituximab or cyclophosphamide.</p><p><strong>Methods: </strong>In this descriptive, post hoc analysis, patients receiving avacopan or a prednisone taper were stratified by age. Key efficacy outcomes included the rate of remission at week 26 and sustained remission at week 52.</p><p><strong>Results: </strong>Of 160 patients aged ≥65, 109 were aged 65-74 and 51 were ≥75. Remission at week 26 was achieved in 71.7% vs 69.4% of patients aged 65-74 and 73.1% vs 72.0% aged ≥75 in the avacopan vs prednisone taper groups, respectively. Sustained remission at week 52 was observed in 65.0% vs 55.1% of patients aged 65-74 and 65.4% vs 56.0% aged ≥75. Relapse rates in the avacopan vs prednisone taper groups were 12.3% vs 18.8% and 3.8% vs 20.8% in the 65-74 and ≥75 subgroups, respectively. Improvements in estimated glomerular filtration rate and health-related quality of life were observed in both treatment groups. Use of avacopan compared with a prednisone taper was associated with a 61% and 49% reduction in mean glucocorticoid dose in the 65-74 and ≥75 subgroups, respectively, and lower glucocorticoid toxicity. The proportions of patients with adverse events were similar between treatment groups within each age subgroup.</p><p><strong>Conclusion: </strong>These data support the efficacy and safety of an avacopan-based regimen to treat patients with GPA or MPA aged ≥65.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"3863-3871"},"PeriodicalIF":4.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557844","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":"Vasculitis associated with VEXAS syndrome.","authors":"Megan M Sullivan, Carolyn Mead-Harvey, Julio C Sartori-Valinotti, Kambiz Kalantari, Yael N Kusne, Mrinal M Patnaik, Abhishek A Mangaonkar, Ronald S Go, Daniel Montes, Kaaren K Reichard, Horatiu Olteanu, Melanie C Bois, Alexander S Hines, Kenneth J Warrington, Matthew J Koster","doi":"10.1093/rheumatology/keae550","DOIUrl":"10.1093/rheumatology/keae550","url":null,"abstract":"<p><strong>Objectives: </strong>To define the prevalence, distribution and characteristics of patients with VEXAS (vacuoles, E1-enzyme, X-linked, autoinflammation, somatic) syndrome who have confirmed vasculitis.</p><p><strong>Methods: </strong>Patients with VEXAS syndrome, verified by positive UBA1 mutation, were included. Chart review was performed to identify patient characteristics and outcomes. Vasculitis diagnosis was based on either histopathology showing vascular inflammation or non-invasive angiography findings. Summary statistics were calculated.</p><p><strong>Results: </strong>Eighty-nine patients met inclusion criteria. All were male with a median age of onset of 66.9 years (interquartile range 60.1, 72.7). Median (interquartile range) follow-up was 3.8 (2.2-5.5) years, during which 21 patients (23.6%) had evidence of vasculitis. Vasculitis subtypes included small vessel vasculitis (19.1%), cutaneous medium vessel vasculitis (2.2%) and large vessel vasculitis (2.2%). No patient had more than one vessel size involved. Histopathology in small vessel vasculitis patients was consistent with cutaneous leukocytoclastic vasculitis in the majority, though one patient had leukocytoclastic peritubular capillaritis on renal biopsy. Cranial symptoms (headache, vision changes or jaw pain) were noted in 18.0%. Two additional patients not experiencing cranial symptoms exhibited large vessel involvement with confirmed carotid thickening on non-invasive angiography; one of these had a positive temporal artery biopsy.</p><p><strong>Conclusion: </strong>VEXAS syndrome manifests as a variable vessel vasculitis in a quarter of patients, with cutaneous small and medium vessel involvement being particularly common. Some patients may have positive ANCA serologies or even renal vasculitis leading to misdiagnosis. Cranial symptoms are common and may mimic GCA, though documented large vessel inflammation is rare.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"3889-3894"},"PeriodicalIF":4.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107069/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142401135","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}