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Reviews

Current and future therapeutic strategies for treating mixed cryoglobulinemia

, MD PhD &
Pages 381-390 | Published online: 13 Feb 2014
 

Abstract

Introduction: Treatment of mixed cryoglobulinemic (MC) vasculitis may target either the viral trigger hepatitis C virus (HCV) or the downstream B-cell arm of autoimmunity. This review will focus on advances in our understanding of the treatment of MC vasculitis.

Areas covered: Aggressive antiviral therapy should be systematically considered for HCV–MC patients. As induction therapy, in mild to moderate MC patients and following rituximab in patients presenting with more severe disease. If failure or contraindication of antiviral therapy or in non-HCV cryoglobulinemia vasculitis, rituximab may be used alone. For patients presenting with the fulminant forms, including peripheral necrosis of extremities, rapidly progressive glomerulonephritis, digestive, cardiac, pulmonary and/or central nervous system involvement, apheresis can have immediate beneficial effects but must be combined with immunosuppression to avoid post-apheresis rebound of MC. High-dose steroids, rituximab, and sometimes cyclophosphamide combination appeared as an effective salvage treatment for refractory MC. In such cases, antiviral therapy should be postponed after the critical phase.

Expert opinion: Rituximab is an effective and safe option for severe MC patients. New antiviral agents would allow eradication of HCV infection in up to 90% of cases and thus increase in the proportion of HCV–MC remission.

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