CLINICAL AND IMMUNOLOGICAL ASPECTS OF WEGENER GRANULOMATOSIS

Wegener's granulomatosis belongs into the group of systemic vasculitis. The disease has to be taken into account as a differential diagnosis of unclear, therapy-resistant infectious processes of the nose, the ear and the upper respiratory tract. The detection of nearly specific anti-neutrophil cytoplasm antibodies against granulocytes (ACPA) allows a serological diagnosis additionally to the clinical picture and the morphological findings. Own data and data of the literature show a high specificity of the ACPA-test in high-activity stage of the disease, whereas the antibodies are not detectable after immunosuppressive induced remission of Wegener's granulomatosis. The therapy of choice is the long-term application of cyclophosphamide and prednisone under clinical and serological control. The immunosuppressive therapy improved and changed the prognosis of Wegener's granulomatosis dramatically. Before the immunosuppressive area the patients died within 6 months, whereas under the modern therapeutical regime long-term remissions, maybe healing of Wegener's granulomatosis can be revealed.