[ANCA-associated vasculitis (Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis). 3. Therapeutic procedure].

Recent studies on the long-term clinical course of ANCA-associated vasculitides have revealed considerable variation in the clinical severity of the disease. The most effective standard therapy (i.e., daily cyclophosphamide + prednisolone, "FAUCI-scheme") is associated with high treatment-related morbidity and mortality as well as a high incidence of relapse. On the other hand, less toxic therapeutic strategies (e.g., monthly bolus of cyclophosphamide or weekly low-dose methotrexate) are being pursued with remarkable success in non-life threatening disease. Intractable cases (under daily cyclophosphamide + prednisolone) may profit from additional high dose IVIG therapy or from monoclonal antibodies against CD4 and CDw52 molecules. Plasmapheresis can be effective in combination with the standard therapy in dialysis patients with necrotizing glomerulonephritis (in Wegener's granulomatosis or microscopic polyarteritis). On the other hand, Wegener's granulomatosis restricted to the upper and/or lower respiratory tract ("Initial WG") responds to trimethoprimsulfamethoxazole. In future, therefore, an individualized strategy adapted to extension and activity has to be developed for the treatment of each patient with ANCA-associated vasculitis.