ABSTRACT
Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms. Diverse processes may coexist in the pathogenesis of SAPH, and there is an overlap with mechanisms of pulmonary arterial hypertension (PAH). This has encouraged the study of PAH-specific therapeutic agents in the treatment of SAPH. In small series, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have been shown to improve hemodynamics, functional status, and outcomes. This article reviews the most recent data available in the epidemiology, pathophysiology, diagnosis, and treatment of SAPH.
KEYWORDS
Sarcoidosis - pulmonary hypertension - treatment
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Roxana SulicaM.D.
Division of Pulmonary and Critical Care Medicine, Beth Israel Medical Center
10 Union Square East, Ste. 2A, New York, NY 10003
eMail: RSulica@chpnet.org
