A rare case of sarcoidosis-associated pulmonary hypertension in a patient exposed to silica. (9th February 2016) - Bourlier, Delphine
Pulmonary hypertension (PH) is a significant and well-recognised complication of sarcoidosis that markedly increases mortality. The current European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines classify sarcoidosis-associated PH in group 5 [1]. Indeed, the pathophysiology is poorly understood and multiple mechanisms may contribute to its pathogenesis. The extrinsic compression of the large pulmonary arteries and/or veins by fibrosing mediastinitis is an uncommon mechanism of sarcoidosis-associated PH. Recognition of PH complicating sarcoidosis-associated fibrosing mediastinitis may be challenging, as the radiological features may suggest other causes of PH. Here, we report the case of a patient with sarcoidosis-associated fibrosing mediastinitis with resultant PH caused by exposure to silica and mimicking chronic thromboembolic pulmonary hypertension (CTEPH). Mechanism of sarcoidosis-associated pulmonary hypertension may be challenging and requires high-quality imaging http://ow.ly/UvJPh