Wegener's granulomatosis.

Wegener's granulomatosis is characterized by a necrotizing granulomatous vasculitis which can be found in both the upper and lower respiratory tracts and with either focal or proliferative glomerulonephritis. However, any organ system can be affected by the disease. Over the past 17 years, 47 patients with histologically proven Wegener's granulomatosis have been treated at the National Institute of Allergy and Infectious Disease. Since 1972, patients with head and neck manifestations have been managed in collaboration with the Department of Otolaryngology, National Naval Medical Center. Experiences with these patients have shown that all have had some degree of respiratory tract involvement, with 42/47 having disease in the nose or paranasal sinuses. An effective therapeutic regimen is possible with immunosuppressants (particularly cyclophosphamide) and locally supportive measures. As a result of such therapy, more than 80% of the patients treated have experienced long-term remissions. The clinical implications of this therapy are discussed, and a protocol for patient management presented.