Cutaneous Vasculitis in Primary Sjögren Disease

Importance Cutaneous vasculitis (CV) is a heterogenous and severe complication of primary Sjögren disease, often indicating systemic involvement and poor prognosis. Understanding its characteristics and outcomes is essential for patient management. Objective To describe the clinical features, management, and prognosis of different CV types in patients with Sjögren disease. Design, Setting, and Participants This retrospective multicenter cohort study included patients with Sjögren 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 Sjögren disease but without CV. Data were collected and analyzed between March 2023 and March 2025. Exposure Presence of CV classified using the Chapel Hill classification dermatological addendum. Main Outcomes and Measures Demographics, clinical characteristics, immunological parameters, and received treatments were recorded. Primary outcomes were lymphoma occurrence and mortality risk, as well therapeutic response. Results Among 54 patients with CV and Sjögren 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 Sjögren disease and CV had a higher lymphoma incidence (12 of 54 [13%] vs 4 of 108 [4%]; P = .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 = .005), higher ESSDAI (EULAR Sjögren Syndrome Disease Activity Index) scores (median [IQR], 15 [12-23]; P = .005), and more frequent kidney involvement (7 of 24 patients [29%] vs 1 of 25 patients [4%]; P = .02) and peripheral nervous system involvement (15 of 24 patients [63%] vs 3 of 25 patients [12%]; P < .001). Rituximab-based therapy showed no survival benefit for patients with type II cryoglobulinemic vasculitis compared to other treatments. Conclusions and Relevance In this cohort study, among CV subtypes in patients with Sjögren 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.