Thoracic manifestations of Wegener granulomatosis: diagnosis and course

A combination of cytotoxic and corticosteroid therapy has dramatically improved the long-term survival of patients with Wegener granulomatosis. With extended survival, patients now experience diverse cardiopulmonary abnormalities that represent primary or secondary manifestations of the disease or that result from diagnosis and treatment. To evaluate these abnormalities, the authors reviewed the medical histories and chest radiologic findings of 19 patients with the histologic diagnosis of Wegener granulomatosis. In these patients thoracic images demonstrated parenchymal nodules or consolidations with cavitation, diffuse interstitial disease, mediastinal or hilar adenopathy, and isolated stenoses of the larynx or tracheobronchial tree. Intrathoracic relapse occurred in 18 cases; in one-third of these patients, findings at relapse differed from those at initial presentation. Complications from diagnosis or therapy occurred in nine patients. Pulmonary infection was the most frequent complication causing morbidity and was often clinically indistinguishable from the primary disease; it complicated relapse in five patients. The successful radiologic follow-up of patients with Wegener granulomatosis requires a consideration of the varied thoracic manifestations of both the primary disease and the complications of its treatment.