ABSTRACT
The small-vessel vasculitides are suitable paradigms for new forms of therapy in autoimmune disease. They are treatment-responsive and many components of the immune and inflammatory systems are involved in their pathogenesis. In particular, there is convincing evidence of T-cell and autoantibody contributions to injury. Today's drugs are usually effective-a remission rate of 93% was achieved in a recent study of generalized systemic vasculitis-but concern to avoid relapse necessitates prolonged therapy, and drug toxicity is almost universal (1,2). Even in ·'expert" centers, severe adverse effects of therapy in systemic vasculitis occur in one-quarter of patients, and there is an appreciable treatment-related mortality, especially in the elderly (2,3). Current therapy rests on the combination of steroids with cytotoxic drugs and regimens continue to be refined in order to maintain efficacy and minimize adverse effects (4,5). Newer approaches to treatment have arisen from a growing understanding of the pathogenesis of vasculitis and from the development of immunotherapeutic drugs with specificity for circulating agents or membrane receptors. The use of synergistic combinations of existing and newer immunosuppressive drugs allows improved efficacy with reduced toxicity. Finally, more sophisticated subgrouping at diagnosis according to prognostic markers and risk for drug toxicity will facilitate "tailored" protocols (5).
