Abstract
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.
Keywords
precapillary pulmonary hypertension - bosentan - epoprostenol - echocardiography -
right heart catheterization
