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-alpha (IFN-alpha) has been shown to be effective in treatment-resistant
Churg-Strauss syndrome, and IFN-alpha 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 Behçet's syndrome; severe refractory Behçet's syndrome
uveitis responded to treatment with IFN-alpha.