Source Department of Internal Medicine, Groupe Hospitalier Pitie-Salpetriere, Assistance Publique Hopitaux de Paris (AP-HP), Universite Pierre et Marie Curie, Paris 6, Paris, France;

Data on the clinical spectrum and therapeutic management of non-infectious mixed cryoglobulinemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking. We analyzed data from 242 patients with non-infectious mixed CryoVas included in the French multicenter CryoVas survey. Baseline manifestations were purpura (75%), peripheral neuropathy (52%), arthralgia or arthritis (44%), glomerulonephritis (35%), cutaneous ulcers (16%) and cutaneous necrosis (14%). A connective tissue disease was diagnosed in 30% and B-cell non-Hodgkin lymphoma in 22%, whereas the CryoVas was considered to be essential in 48%. Using Cox-marginal structural models, rituximab plus corticosteroids showed the greater therapeutic efficacy compared to corticosteroids alone and alkylating agents plus corticosteroids to achieve complete clinical, renal and immunological responses and a prednisone dosage < 10 mg/day at 6 months. However, this regimen was also associated with severe infections, particularly when high doses of corticosteroids were used, while death rates did not differ between the therapeutic regimens. The role of each of these strategies remains to be defined in well-designed randomized controlled trials.