Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients

INTRODUCTION: cryoglobulinemia vasculitis (CV) is a systemic vasculitis secondary to circulating immune complex deposition in small blood vessels. In the overwhelming majority of patients, it is associated to hepatitis C virus (HCV) infection, nevertheless it has been observed in a wide variety of diseases and in patients with no underling conditions - "essential cryoglobulinemia". OBJECTIVE: to report a series of patients with non-HCV CV. PATIENTS AND METHODS: a retrospective chart review of non-HCV CV patients was carried out by members of the Minas Gerais Vasculitis Study Group. Demographics, clinical, laboratory data, treatments and follow-up are reported. RESULTS: nine patients, two men and seven women, aged a 55.3 ± 17 years were studied. In three patients, CV was attributed to connective tissue disease and in one to a monoclonal gammopathy of undetermined significance (MGUS). Five patients presented essential cryoglobulinemia. Four patients presented bone marrow lymphoid infiltrates, but none developed overt lymphoma. All patients complained of myalgia/arthralgia. Eight patients (89 percent) presented cutaneous vasculitis; four patients (44 percent) had glomerulonephritis (GN) and six patients (67 percent) had neuropathy. Regarding treatment, six patients responded to steroids and immunosuppressive agents. Three patients were refractory to conventional therapy and therefore were treated with rituximab. After a follow-up of 19.3 ± 14 months, five patients (56 percent) were in partial remission and four patients (44 percent) were in complete remission. CONCLUSION: non-HCV cryoglobulinemia may present itself as a systemic vasculitis. It should not be understood as a simple inflammatory disease, since most patients present evidence of a lymphoproliferative disorders. Treatment should include steroids and immunosuppressive agents. Refractory patients may benefit themselves from rituximab.