Approximately 90% of patients with sarcoidosis have thoracic involvement, of the lungs or mediastinum or both, isolated in half of all cases and associated with one or several extrathoracic sites in the other half. Diagnosis is based on a compatible clinical and radiologic history, the demonstration of noncaseous granulomas, and the ruling out of other causes of granulomatosis. Histologic proof is not necessary in cases of Löfgren syndrome, or when documentation shows a sequence of progression from stage I to stage II, or when the radiography shows the typical latent stage I picture. The radiographic stage has major prognostic value. Pulmonary fibrosis is the principal cause of morbidity and mortality in patients with sarcoidosis, especially when pulmonary hypertension is also present. Approximately half of all patients recover spontaneously in less than 2 years, while the other half require a systemic treatment. The official indications for treatment are: symptomatic stage II-III disease with functional respiratory or progressive involvement, stage IV with signs of persistent activity, granulomatous bronchial involvement with obstructive ventilatory disorders or endobronchial stenosis and extrinsic bronchial compression by adenopathies. Systemic corticosteroid therapy remains the reference treatment for thoracic involvement in sarcoidosis.
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