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

Duong, Heng, T., MD; Bonham, Catherine, A., MD

doi: 10.1097/CPM.0000000000000252
Interstitial, Inflammatory and Occupational Lung Disease
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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.

Section of Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL

H.T.D. was supported by NIH/NHLBI T32 HL007605 Respiratory Biology Training Grant and C.A.B. was supported by NIH/NHLBI Grant K12 HL119995.

Disclosure: The authors declare that they have no conflicts of interest.

Address correspondence to: Catherine A. Bonham, MD, Section of Pulmonary and Critical Care Medicine, University of Chicago, 5841 South Maryland Avenue, Room W652B, MC 6076, Chicago, IL 60637. E-mail: catherine.bonham@uchospitals.edu.

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