Sarcoidosis-associated Pulmonary Hypertension: Pathophysiology, Diagnosis, and Treatment

Clinicians in pulmonary medicine frequently confront the challenge of screening, diagnosis and management of pulmonary hypertension (PH) in sarcoidosis patients who present with unexplained dyspnea. Sarcoidosis associated pulmonary hypertension (SAPH) is most prevalent in patients with pulmonary fibrosis, though it can be independent of airflow obstruction or restriction. SAPH independently associates with significantly increased mortality and decreased functional capacity, outcomes which can be mitigated by early detection and focused treatment. In this review, we discuss the pathophysiology of SAPH, which may resemble pulmonary arterial hypertension as well as secondary causes of PH. We offer a screening algorithm for SAPH, and advocate for detailed assessment of the cause of PH in each patient prior to choice of an individualized treatment plan. We note that treatment of sarcoidosis via immune suppression is typically insufficient to adequately treat SAPH. We discuss secondary causes of SAPH such as left heart disease, sleep disordered breathing, and thromboembolic disease, and the evidence for use of PH-specific therapy in select cases of SAPH. Management of SAPH by clinicians experienced in PH, with early referral to transplantation in refractory cases is advised.