Wegener's granulomatosis. Combined therapy with low-dosage systemic corticosteroids, azathioprine and cyclophosphamide in three patients.

Three patients with Wegener's granulomatosis are reported. All three had skin, nose, sinus, mouth and pulmonary lesions; one had severe renal involvement as well. In two patients combined treatment with low-dosage systemic corticosteroids, azathioprine, and cyclophosphamide resulted in rapid clinical improvement and complete disappearance of the skin, mouth, nasal, sinus and pulmonary lesions. In one of these two patients who had severe renal involvement, proteinuria greatly diminished and arrest of progression of renal insufficiency was observed. In the third patient combined treatment with azathioprine and cyclophosphamide resulted in complete remission of skin, mucous membrane, lung and sinus lesions. The patient with severe renal involvement is alive and ambulant 4 years after the onset of the disease, the other two are completely symptom-free and well 2 1/2 years and 1 year after the onset of their illnesses, respectively. These results confirm previous reports about the effectiveness of immunosuppressive and cytotoxic agents in Wegener's granulomatosis, the use of which seems to have improved the prognosis significantly in a disease previously considered to be fatal within 5 months.