[Diagnosis and therapy in systemic vasculitis with renal involvement]

The diagnosis of Wegener's granulomatosis, microscopic polyarteritis and idiopathic rapidly progressive glomerulonephritis as the main cause of renal involvement in systemic necrotizing vasculitis was revolutionized following the introduction of the ANCA test. The different clinical manifestations of these diseases are described. By means of the ANCA test it could be demonstrated that the pulmonary-renal syndrome was mainly caused by these vasculitides and not by Goodpasture's syndrome. Cyclophosphamide and steroids remain the drugs of choice in the treatment. They can be administered in different ways depending upon the clinical manifestation of the diseases. As therapeutic alternatives cyclosporin, trimethoprim/sulfamethoxazole, intravenous immunglobulins and monoclonal antibodies are discussed.