Sarcoidosis-associated pulmonary hypertension: assessment and management.
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.
Palmero
Veronica
V
Division of Pulmonary and Critical Care Medicine, Beth Israel Medical Center, New York, NY 10003, USA.
Sulica
Roxana
R
D065128:Endothelin Receptor Antagonists; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D044062:Prostaglandins I / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D012507:Sarcoidosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors; D044062:Prostaglandins I