Rituximab for Sjögren syndrome-associated type II mixed cryoglobulinemic cerebral vasculitis.

Mixed cryoglobulinemia (MC) is caused by the precipitation of polyclonal immunoglobulin (Ig)M or IgG below 37°C. This condition is mainly associated with hepatitis C virus (HCV) but also with autoimmune diseases, such as Sjogren syndrome.1 CNS involvement is usually described in MC associated with HCV infection2 and in a recent study, only in 2% of 242 patients with noninfectious MC vasculitis.3 Treatment of the underlying disease, corticosteroids, and immunosuppressive drugs are essential for the management of cryoglobulinemic vasculitis. The use of rituximab, a monoclonal chimeric IgG1κ anti-CD20 antibody targeting B lymphocytes, seems promising both for the treatment of MC manifestations and cerebral vasculitis.4 Herein, we describe 2 cases of cerebral cryoglobulinemic vasculitis associated with Sjogren syndrome successfully treated with rituximab.