{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,10]],"date-time":"2026-07-10T19:42:12Z","timestamp":1783712532218,"version":"3.55.0"},"reference-count":12,"publisher":"American Medical Association (AMA)","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Dermatol"],"published-print":{"date-parts":[[2025,10,1]]},"abstract":"Importance<\/jats:title>Cutaneous vasculitis (CV) is a heterogenous and severe complication of primary Sj\u00f6gren disease, often indicating systemic involvement and poor prognosis. Understanding its characteristics and outcomes is essential for patient management.<\/jats:p><\/jats:sec>Objective<\/jats:title>To describe the clinical features, management, and prognosis of different CV types in patients with Sj\u00f6gren disease.<\/jats:p><\/jats:sec>Design, Setting, and Participants<\/jats:title>This retrospective multicenter cohort study included patients with Sj\u00f6gren disease and CV from pathology departments of 3 university hospitals in Paris, France (2011-2021), and a national case call. Patients met American College of Rheumatology\/European League Against Rheumatism criteria and were matched 1:2 to controls patients with Sj\u00f6gren disease but without CV. Data were collected and analyzed between March 2023 and March 2025.<\/jats:p><\/jats:sec>Exposure<\/jats:title>Presence of CV classified using the Chapel Hill classification dermatological addendum.<\/jats:p><\/jats:sec>Main Outcomes and Measures<\/jats:title>Demographics, clinical characteristics, immunological parameters, and received treatments were recorded. Primary outcomes were lymphoma occurrence and mortality risk, as well therapeutic response.<\/jats:p><\/jats:sec>Results<\/jats:title>Among 54 patients with CV and Sj\u00f6gren disease (median [IQR] age at diagnosis of CV, 42 [27.7-56.0] years; 49 [91%] female), CV was more frequently classified as cryoglobulinemic vasculitis (29 patients [57%]) or hypergammaglobulinemic vasculitis (15 patients [28%]). Compared to controls, patients with Sj\u00f6gren disease and CV had a higher lymphoma incidence (12 of 54 [13%] vs 4 of 108 [4%]; P<\/jats:italic>\u2009=\u2009.04). Compared to other types of CV, type II cryoglobulinemic vasculitis was associated with increased mortality or lymphoma risk (hazard ratio, 6.8; 95% CI, 1.8-25.5; P<\/jats:italic>\u2009=\u2009.005), higher ESSDAI (EULAR Sj\u00f6gren Syndrome Disease Activity Index) scores (median [IQR], 15 [12-23]; P<\/jats:italic>\u2009=\u2009.005), and more frequent kidney involvement (7 of 24 patients [29%] vs 1 of 25 patients [4%]; P<\/jats:italic>\u2009=\u2009.02) and peripheral nervous system involvement (15 of 24 patients [63%] vs 3 of 25 patients [12%]; P<\/jats:italic>\u2009&amp;lt;\u2009.001). Rituximab-based therapy showed no survival benefit for patients with type II cryoglobulinemic vasculitis compared to other treatments.<\/jats:p><\/jats:sec>Conclusions and Relevance<\/jats:title>In this cohort study, among CV subtypes in patients with Sj\u00f6gren disease, only type II cryoglobulinemic vasculitis was associated with poor prognosis. Early recognition and monitoring for systemic complications, particularly lymphoma, are essential in these patients. Further research is needed to evaluate optimal treatment strategies for improving outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1001\/jamadermatol.2025.2665","type":"journal-article","created":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T15:00:38Z","timestamp":1754492438000},"page":"1057","source":"Crossref","is-referenced-by-count":3,"title":["Cutaneous Vasculitis in Primary Sj\u00f6gren Disease"],"prefix":"10.1001","volume":"161","author":[{"given":"Paul","family":"Breillat","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine Interne, Centre de R\u00e9f\u00e9rence Maladies Syst\u00e9miques Autoimmunes et Autoinflammatoires Rares d\u2019Ile de France, de l\u2019Est et de l\u2019Ouest, H\u00f4pital Cochin, Assistance Publique-H\u00f4pitaux de Paris (AP-HP)-CUP, Universit\u00e9 de Paris Cit\u00e9, Paris, France"},{"name":"INSERM, U970, Paris Centre de Recherche Cardiovasculaire (PARCC), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00e9ronique","family":"Le Guern","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, Centre de R\u00e9f\u00e9rence Maladies Syst\u00e9miques Autoimmunes et Autoinflammatoires Rares d\u2019Ile de France, de l\u2019Est et de l\u2019Ouest, H\u00f4pital Cochin, Assistance Publique-H\u00f4pitaux de Paris (AP-HP)-CUP, Universit\u00e9 de Paris Cit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"d\u2019Humi\u00e8res","sequence":"additional","affiliation":[{"name":"Department of Physiology, FHU SENEC, Henri Mondor Hospital, Assistance Publique H\u00f4pitaux de Paris, INSERM IMRB U955, Universit\u00e9 Paris Est, Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxime","family":"Battistella","sequence":"additional","affiliation":[{"name":"Department of Pathology, AP-HP, Saint-Louis Hospital, 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