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Cryoglobulinemic vasculitis in primary Sjögren's syndrome: Clinical presentation, association with lymphoma and comparison with hepatitis c-related disease

Seminars in Arthritis and Rheumatism Jul 31, 2020

Argyropoulou OD, Pezoulas V, Chatzis L, et al. - This study was intended to present the clinical spectrum of cryoglobulinemic vasculitis (CV) in primary Sjögren's syndrome (pSS), investigate its relation to lymphoma, and distinguish the differences with hepatitis C virus (HCV) related CV. In this study, those who had been assessed for cryoglobulins and fulfilled the 2011 classification criteria for CV were distinguished retrospectively from a multicentre study population of consecutive pSS patients. Researchers compared pSS-CV patients with pSS patients without cryoglobulins (1:2) and HCV-CV patients (1:1). They examined clinical, laboratory, and outcome features. They used a data-driven logistic regression model for pSS-CV patients and their pSS cryoglobulin negative controls to distinguish independent features correlated with lymphoma. This study investigated 1083 pSS patients for cryoglobulins. In comparison with HCV related CV, pSS-CV has a severe clinical course, overshadowing the typical clinical manifestations of pSS and higher risk for early lymphoma development. It was noted that though infrequent, pSS-CV constitutes a distinct severe clinical phenotype of pSS.

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