Current Treatment of Cryoglobulinemic Vasculitis

For patients with cryoglobulinemic vasculitis, we consider treatment of the underlying disorder (if present) to be first-line therapy. For hepatitis C virus (HCV)-associated cryoglobulinemic vasculitis, antiviral therapy directed at HCV should be considered first-line treatment for patients who are candidates. Interferon-free direct acting antiviral regimens have been shown to be very effective in the treatment of chronic HCV infection and preliminary results of the use of these regimens are promising for the treatment of HCV-associated cryoglobulinemic vasculitis. Close collaboration with a gastroenterologist or hepatologist is needed, given the complexity in choosing from the various treatment options. Patients with severe life- or organ-threatening manifestations of cryoglobulinemic vasculitis may benefit by treatment with rituximab in addition and potentially prior to the initiation of antiviral therapy. We also recommend treatment with rituximab in patients who have a contraindication to or who have failed antivirals. Plasmapheresis, in conjunction with immunosuppression, can be used as adjunctive therapy to antiviral and/or rituximab therapy for patients with severe organ- or life-threatening disease. Cyclophosphamide use is best reserved for patients with severe disease who are unable to be treated with antiviral or rituximab therapy. The role of glucocorticoid use remains poorly defined, with some studies supporting moderate or high-dose prednisone use to treat active disease.