Treatment options for mixed cryoglobulinemic vasculitis
Removal of the antigenic stimulus is the primary goal. In HCV-associated mixed cryoglobulinemic vasculitis, this involves use of standard AV regimens, whereas in malignancy, other infections, and autoimmune diseases, appropriate treatment of these conditions is warranted. Immunosuppression is used to directly control the vasculitis and associated tissue damage. Most data regarding treatment are derived from studies of HCV-associated mixed cryoglobulinemic vasculitis:
• Glucocorticoids: high-dose steroids should be used in patients with severe manifestations (e.g., neurologic, renal, or diffuse vasculitis), with short courses of low to intermediate doses for minor flares. Steroids should be tapered off quickly, and there is no role for chronic therapy.
• Plasma exchange: although there is no controlled data supporting its use, expert opinion suggests a role in severe, life-threatening disease. It is the treatment of choice for hyperviscosity syndrome.
• Cyclophosphamide: it is frequently employed in combination with plasma exchange to treat severe disease. It is not recommended for use as monotherapy.
• Other immunosuppressants: use of azathioprine, methotrexate, cyclosporine, and other immunosuppressive agents is anecdotal, and no consensus recommendations can be made.
• Colchicine: it may have favorable effects on pain, weakness, purpura, and leg ulcers. The standard dose is 1 mg/day.
• Low-antigen diet: older data suggests an improvement in purpura and arthralgias within 4 to 8 weeks, as well as favorable effect on laboratory abnormalities.
• AVs and RTX