Semin Respir Crit Care Med 2017; 38(04): 450-462
DOI: 10.1055/s-0037-1603767
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Sarcoidosis-Associated Pulmonary Hypertension

Oksana A. Shlobin
Pulmonary Vascular Disease Program, Inova Fairfax Hospital, Falls Church, Virginia
Robert P. Baughman
Department of Medicine, University of Cincinnati Medical Center, Cincinnati, Ohio
› Author Affiliations
Further Information

Publication History

Publication Date:
27 July 2017 (online)


Sarcoidosis-associated pulmonary hypertension (SAPH) is found in 5 to 20% of sarcoidosis patients. Elevated pulmonary artery pressure may be due to multiple factors, including vasculocentric, parenchymal, and mechanical, as well as comorbidities such as cardiac sarcoidosis and sleep apnea. Most SAPH patients have fibrotic lung disease, but SAPH may be present in those without advanced parenchymal lung disease. Several features have been shown to suggest SAPH, including reduced DLCO, shortened 6-minute walk distance, with or without desaturation, and the presence of increased pulmonary artery to aorta ratio on CT scanning. Echocardiography remains an important tool for the evaluation of SAPH but may both over- or underestimate the severity of pulmonary artery pressure. Right heart catheterization remains the definitive test to make the diagnosis. There have been several reports on the value of different modalities of treating SAPH. These include prospective clinical trials and one double-blind placebo-controlled randomized trial. Evidence-based guidelines for treatment of SAPH are discussed in this review.