Sarcoidosis-associated fibrosing mediastinitis with resultant pulmonary hypertension: a case report and review of the literature

Abstract

Pulmonary hypertension, a common manifestation of advanced sarcoidosis, is thought to result from fibrosis with chronic hypoxia and destruction of small vessels, extrinsic compression of pulmonary arteries, or granulomatous vasculitis. We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on exertion and was found to have pulmonary artery enlargement, pulmonary venous compression, and mediastinal soft tissue enhancement on magnetic resonance imaging. Pulmonary hypertension was confirmed by right heart catheterization and sarcoidosis was diagnosed by histologic examination of tissue obtained at mediastinoscopy. Treatment with steroids resulted in decreased pulmonary artery pressures as well as symptomatic improvement. While pulmonary hypertension is a common complication of sarcoidosis, fibrosing mediastinitis is an unusual etiology that should be considered by clinicians.