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Cryoglobulinemic vasculitis

Ferri, Clodoveo; Mascia, Maria Teresa

Current Opinion in Rheumatology: January 2006 - Volume 18 - Issue 1 - p 54–63
Vasculitis syndromes

Purpose of review: Cryoglobulinemic vasculitis is an immune-complex-mediated systemic vasculitis involving small–medium-sized vessels. A causative role of hepatitis C virus in over 80% patients has been definitively established, with heterogeneous geographical distribution. This review focuses on recent etiopathogenetic, clinico-diagnostic, and therapeutical studies.

Recent findings: Hepatitis C virus cannot be integrated into the host genome; it may exert a chronic stimulus to the immune system. The interaction between hepatitis C virus envelope protein E2 with B-cell CD81 receptor may increase the frequency of VDJ rearrangement in antigen-reactive B lymphocytes. One consequence is the activation of various protooncogenes, including antiapoptotic Bcl-2. The extended B-cell survival is responsible for autoantibody and immune-complex production, including mixed cryoglobulins; some malignancies, mainly B-cell lymphomas, may complicate cryoglobulinemic vasculitis. Environmental or viral/host genetic cofactors should be relevant in the pathogenesis of hepatitis C virus-related diseases. Cryoglobulinemic vasculitis may overlap with other diseases (systemic vasculitides, Sjögren's syndrome, autoimmune hepatitis, lymphoma), which should be carefully considered for a correct diagnosis and treatment. Cumulative survival of cryoglobulinemic vasculitis is significantly lower compared with the general population. Therapeutic strategies for cryoglobulinemic vasculitis include etiologic (antiviral), pathogenetic (cyclophosfamide, rituximab), or symptomatic (steroids, plasmapheresis) treatments, which should be tailored to the individual patient according to the severity/activity of clinical symptoms.

Summary: Cryoglobulinemic vasculitis represents a crossroads among autoimmune and lymphoproliferative disorders; as hepatitis C virus infection is the major causative factor, cryoglobulinemic vasculitis is an important model for etiopathogenetic studies of virus-related diseases.

Chair and Rheumatology Unit, Department of Internal Medicine, University of Modena e Reggio Emilia, Medical School, Modena, Italy

Correspondence to Professor Clodoveo Ferri MD, Reumatologia, Università di Modena e Reggio Emilia, Policlinico di Modena, Via del Pozzo, 71, 41100 Modena, Italy Tel: +39 059 4222279; fax: +39 059 4223260; email:

© 2006 Lippincott Williams & Wilkins, Inc.