Sarcoidosis-Associated Pulmonary Hypertension: An Updated Review and Discussion of the Clinical Conundrum

Abstract

Pulmonary hypertension (PH) is a life-threatening disease with complex pathophysiology. The World Health Organization has classified PH in 5 groups according to etiology, the fifth of which corresponds to PH due to unknown or multiple mechanisms; including sarcoidosis-associated PH (SAPH). Although this system has been used to guide treatment recommendations according to each group, it does not provide much insight into the heterogeneous group 5. Furthermore, pulmonary vasodilators have been contraindicated for patients in this cluster which represents a challenge for the management of SAPH which can sometimes improve with these PH-directed drugs. In this review, we discuss the classification of SAPH; as well as the evidence behind the use of pulmonary vasodilator, invasive procedures, and lung transplantation for treating SAPH; and the little that is known about his disease in the setting of cardiac sarcoidosis.