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


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Abstract

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, although it can be independent of airflow obstruction or restriction. SAPH is independently associated with significantly increased mortality and decreased functional capacity, outcomes that can be mitigated by early detection and focused treatment. In this review, we discuss the pathophysiology of SAPH, which may resemble pulmonary arterial hypertension and secondary causes of PH. We offer a screening algorithm for SAPH and advocate for detailed assessment of the cause of PH in each patient before choice of an individualized treatment plan. We note that treatment of sarcoidosis by immune suppression is typically insufficient to treat SAPH adequately. We discuss the 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.

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