Cryoglobulinaemic Vasculitis

Abstract

Cryoglobulinaemic vasculitis (CV) is a systemic vasculitis affecting small and medium-sized vessels that is caused by immune complexes of cryoprecipitating immunoglobulins and is sustained by benign B-cell clonal proliferation. Palpable purpura is the clinical hallmark of the disease; other classic symptoms are asthenia and arthralgia. The clinical diagnosis of CV must be confirmed by the detection of cryoglobulins. The most frequent pathological trigger is hepatitis C virus (HCV) and it is likely that the prevalence of HCV infection in different geographical areas is decisive in determining the local CV incidence. Systemic autoimmune diseases are probably the second leading cause of CV. Other associated conditions include malignancies and chronic liver disorders. The biochemical mechanisms of cryoprecipitation are not fully understood and this may be different in monoclonal and mixed cryoglobulins. The therapeutic approach to CV should have three objectives: to remove the causes (HCV or other underlying diseases); to block the pathogenetic mechanisms (suppressing B-lymphocyte proliferation); and to reduce the symptoms, including pain.