ABSTRACT
Wegener’s granulomatosis usually presents with underlying necrotizing granuloma tous inflammation and small-and medium-vessel vasculitis involving both the airways and the lung parenchyma. This process is reflected on the chest radiograph as nodules or masses that undergo cavitation. If lesions appear only in the lung or upper airways, this is referred to as limited Wegener’s granulomatosis. If this phase remains untreated, most patients will progress to a more generalized phase of the disease in which the kidneys are involved. The limited disease is associated with very few constitutional symptoms. Generalized disease, on the other hand, is usually associated with malaise, fevers, night sweats, weight loss, and sometimes migratory arthralgias. Pulmonary symptoms include cough, dyspnea, and hemoptysis. Because of tracheobronchial involvement, the symptoms may mimic asthma. The airway obstruction follows acute inflammation, mucosal ulcer ation, and eventually stenosis. It is most common in the subglottic area. Bronchial stenosis may cause lobar atelectasis and postobstructive pneumonia. Other manifestations include a leukocytoclastic vasculitis of the skin, which presents as palpable purpura, central or peripheral nervous system involvement, sinusitis, and glomerulonephritis. The presence of glomerulonephritis adversely affects outcome. The diagnosis of Wegener’s granuloma tosis is established by the presence of serum antiproteinase 3 antibody (c-ANCA) with a compatible clinical picture. If a biopsy is necessary, a lung biopsy is recommended. The renal biopsy rarely if ever shows necrotizing granulomatous inflammation.
