Cutaneous Vasculitis in Primary Sjögren Disease.

Area of Science:

• Rheumatology
• Dermatology
• Immunology

Background:

• Cutaneous vasculitis (CV) is a severe complication of primary Sjögren disease, often signaling systemic involvement and a poor prognosis.
• Understanding CV characteristics and outcomes is vital for managing Sjögren disease patients.

Purpose of the Study:

• To describe the clinical features, management, and prognosis of different CV types in patients with Sjögren disease.
• To identify specific CV subtypes associated with adverse outcomes in Sjögren disease.

Main Methods:

• Retrospective, multicenter cohort study of Sjögren disease patients with CV (2011-2021).
• Classification of CV using the Chapel Hill classification dermatological addendum.
• Matched comparison with Sjögren disease patients without CV, analyzing demographics, clinical data, and outcomes like lymphoma and mortality.

Main Results:

• Type II cryoglobulinemic vasculitis (57%) and hypergammaglobulinemic vasculitis (28%) were the most frequent CV types.
• Sjögren disease patients with CV had a higher incidence of lymphoma compared to controls (13% vs 4%).
• Type II cryoglobulinemic vasculitis was significantly associated with increased mortality or lymphoma risk (HR 6.8), higher ESSDAI scores, and more frequent kidney and peripheral nervous system involvement.

Conclusions:

• Type II cryoglobulinemic vasculitis is the primary CV subtype linked to poor prognosis in Sjögren disease.
• Early recognition and monitoring for systemic complications, especially lymphoma, are essential for patients with CV and Sjögren disease.
• Further research is needed to establish optimal treatment strategies for improving outcomes in these patients.