Von der induktionstherapie bis zur remissionserhaltung: Wegener'sche granulomatose therapie heute und morgen

The clinical presentation of Wegener's granulomatosis (WG) has changed enormously during the last 20 years due to the introduction of new therapeutic and diagnostic approaches. Besides the acute life threatening rapidly progressive disease courses milder forms restricted to the upper respiratory tract are diagnosed more frequently because of better diagnosis. Besides the histology - initially mainly from intranasal tissue - the determination of ANCA (Anti-Neutrophil-Cytoplasmic-Autoantibodies) is a laboratory test with results available in a few hours. Therapy of WG is conformed to the extension and activity of the disease. Only in generalized severe forms the strong immunosuppression with cyclophosphamide and prednisolone is necessary. In less severe forms cyclophosphamide pulse therapy or when there are no renal manifestations methotrexate or azathioprine can be used. After the so called induction therapy with cyclophosphamide therapy is switched to a milder so called remission maintaining therapy with azathioprine, methotrexate and recently also leflunomide or mycophenolate mofetil. In the so called refractory cases with continuously active disease despite cyclophosphamide and prednisolone among others TNFα blocking substances which are applied successfully in other rheumatic diseases are used.