Wegener's granulomatosis with delayed pulmonary and colonic involvement

We have described a patient with Wegener's granulomatosis whose renal manifestations preceded the pulmonary findings by six years. The disease remained undiagnosed because renal transplantation required the institution of azathioprine and prednisone therapy. After immunosuppressive (azathioprine) therapy was discontinued, pulmonary, bowel, and cutaneous vasculitis promptly appeared and the diagnosis of WG was finally made. Autopsy showed complete disease remission induced by cyclophosphamide. This case emphasizes the need for intensive follow-up of patients who have glomerulonephritis of undetermined etiology. We believe the diagnosis of WG should be considered in such patients even if objective pulmonary disease is not present initially. In addition to delayed pulmonary manifestations in WG, we have described heretofore unreported colonic vasculitis with associated multiple bowel wall perforations.