New concepts in treatment protocols for severe systemic vasculitis

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Abstract
Glucocorticosteroids (GCs) are the drug of choice in all clinical types of giant cell arteritis (GCA); a study delineated that an unexpectedly high percentage of patients required long-term GCs, with the consequence of significant complications attributable to GC therapy. Azathioprine and methotrexate are recommended as GC-sparing drugs. Cyclosporin A was found to confer no additive effect versus GC treatment alone. Depot GCs intramuscularly every 3 weeks decreased the cumulative GC dose and were associated with fewer bone fractions compared with daily oral GCs. Pulse cyclophosphamide has been shown to be as effective as the standard therapy in necrotizing vasculitides; however, an alarmingly high rate of infections was observed in this study in both arms possibly related to the high dosage of GCs. New drugs such as mycophenolate mofetil and leflunomide appear as alternatives as maintenance therapy in antineutrophil cytoplasm autoantibody-associated vasculitides in pilot studies. Interferon-α (IFN-α) has been shown to be effective in treatment-resistant Churg-Strauss syndrome, and IFN-a or ribavirin can be used successfully in essential mixed cryoglobulinemia (induced by hepatitis C virus). Thalidomide was shown to be effective for treating oral and genital ulcers and follicular lesions in Behpet's syndrome; severe refractory Behget's syndrome uveitis responded to treatment with IFN-α.