Patients may present with systemic inflammatory symptoms, such as fever, malaise, arthralgias, and weight loss. Upper airway complaints such as sinus pain, purulent or bloody nasal discharge, nasal mucosal ulceration, and serous otitis media are common.
Pulmonary manifestation includes hemoptysis, cough, chest discomfort, and dyspnea.
In most cases, the extrarenal disease precedes the onset of renal disease. Although the cause of the disease is not known, it is felt to represent an antigen triggered immunologic reaction. It is postulated that the necrotic lung granulomas are related to ischemia from the nearby vasculitis. The blood vessel wall is usually involved early with resultant hemorrhage, thrombotic occlusion, and lung infarction. Pulmonary Wegener granulomatosis is characterized by a necrotizing vasculitis that affects blood vessels of all sizes.