Cutaneous Vasculitis in Primary Sjögren Disease

What are the main features, management, and prognosis of cutaneous vasculitis (CV) in primary Sjögren disease?This cohort study included 54 patients with Sjögren disease and CV, mainly cryoglobulinemic vasculitis and hypergammaglobulinemic vasculitis. Compared to controls with Sjögren disease but without CV, patients with Sjögren disease and CV had a higher lymphoma risk (13% vs 4%), and type II cryoglobulinemic vasculitis was associated with increased mortality/lymphoma risk, kidney involvement, and peripheral nerve involvement, while rituximab showed no survival benefit for patients with type II cryoglobulinemic vasculitis compared to patients with other treatment.Among patients with CV, only type II cryoglobulinemic vasculitis was associated with poor prognosis in patients with Sjögren disease.

Corresponding Author: Paul Breillat, MD, INSERM, U970, Paris Centre de Recherche Cardiovasculaire (PARCC), 56 rue Leblanc, 75015 Paris, France (paul.breillat@inserm.fr).

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