A rare case of sarcoidosis-associated pulmonary hypertension in a patient exposed to silica. (9th February 2016)

• Record Type: Journal Article
• Authors: Bourlier, Delphine; O'Connell, Caroline; Montani, David; Savale, Laurent; Seferian, Andrei; Parent, Florence; Humbert, Marc; Simonneau, Gérald; Sitbon, Olivier; Jaïs, Xavier
• Is Part Of: European respiratory review. Volume 25:Number 139(2016)
• Page Start: 93
• Page End: 96
• Publication Date: 2016-02-09
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases; Respiratory organs -- Diseases; Respiratory organs -- Diseases -- Treatment; Electronic journals; Periodical; Periodicals; Periodicals; 616.2
• DOI: 10.1183/16000617.0073-2015
• Languages: English
• ISSNs: 0905-9180

Abstract

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