[Therapy of primary systemic vasculitis]

The treatment of systemic vasculitides has changed during the past ten years from a uniform immunosuppressive regimen consisting of cyclophosphamide and prednisolone to a stage- and activity-adapted, more differentiated immunosuppressive and immunomodulating therapy. Due to the low incidence of these diseases, the assessment of these regimens by prospective, randomized, controlled trials has only been enabled through multicenter cooperation. In primary systemic vasculitides of small- or medium-sized vessels induction of remission therapy comprises methotrexate in early generalized disease without significant renal insufficiency, cyclophosphamide in full-blown generalized disease and additional plasma exchanges in cases of rapidly progressive glomerulonephritis if oliguric, each in conjunction with oral prednisolone. After achievement of remission patients are switched to a less potent maintenance of remission therapy with better long-term tolerability, such as azathioprine. Glucocorticoids are the mainstay for large vessel vasculitides, supplemented by azathioprine or methotrexate in cases of critical organ perfusion or demand for high steroid doses. Therapy for secondary vasculitides relies essentially on the treatment of the underlying disease, which means virus elimination in cases of hepatitis B or C associated vasculitides. Immunosuppression in these diseases is reserved for organ- or life-threatening manifestations only.