Cryoglobulinemia vasculitis: an update.

Objective: Cryoglobulinemia vasculitis (CryoVas) is a small-vessel vasculitis associated with chronic infections [in particular hepatitis C virus, (HCV)], autoimmune disorders and B-cell lymphoproliferative disorders. The most recent studies on its diagnosis, prognosis and therapeutic management are reviewed here.

Results: Large series of patients with HCV-positive and negative mixed CryoVas and patients with monoclonal type I CryoVas have described the presentation and the prognosis of patients with CryoVas in the era of HCV screening. European experts in the field of CryoVas developed new classification criteria for its diagnosis. Finally, French, Italian and North American clinical studies demonstrated that rituximab-based regimens were highly effective in comparison with corticosteroids alone or other immunosuppressive agents-based therapy. However, rituximab seems to be associated with an increased risk of severe infections in a subset of patients.

Conclusions: Recent studies identified prognostic factors of survival and demonstrated that rituximab is highly effective but remains associated with severe infections in a subset of patients. These results could support individual therapeutic stratification according to the clinical pattern and associated comorbidities.