Wegener's granulomatosis: current trends in diagnosis and management

To provide an update on diagnostic methods and treatment options for Wegener's granulomatosis and to review common head and neck manifestations of the disease.

Recent advances have been made in the systemic treatment of Wegener's granulomatosis, including the introduction of investigational immunosuppressive agents such as etanercept, leflunomide and deoxyspergualin. Surgical options remain indicated in selected complications of Wegener's granulomatosis such as saddle nose deformity and subglottic stenosis.

Wegener's granulomatosis is an idiopathic, systemic vasculitis characterized by the formation of necrotizing granulomas of the respiratory tract in addition to focal or proliferative glomerulonephritis. Diagnosis is made by a combination of physical examination, laboratory studies and tissue biopsy. Head and neck manifestations are abundant and varied; common sites of involvement include the middle ear, nose and sinuses and subglottis. The mainstay of treatment remains systemic therapy using a combination of glucocorticoids and immunosuppressants. The otolaryngologist plays a key role in the diagnosis and treatment of head and neck complications of the disease. A surgical role exists for the management of nasal and sinus disease as well as laryngeal and tracheal disease.