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Original Research: PULMONARY HYPERTENSION |

Treatment of Sarcoidosis-Associated Pulmonary Hypertension: A Two-Center Experience

Christopher F. Barnett, MD, MPH; Eric J. Bonura, MD; Steven D. Nathan, MD, FCCP; Shahzad Ahmad, MD; Oksana A. Shlobin, MD; Kwabena Osei, MD, MPH; Ari L. Zaiman, MD; Paul M. Hassoun, MD, FCCP; David R. Moller, MD; Scott D. Barnett, PhD; Reda E. Girgis, MB, BCh, FCCP
Author and Funding Information

*From the Critical Care Medicine Department (Dr. C.F. Barnett), Clinical Center, National Institutes of Health, Bethesda, MD; the Department of Internal Medicine (Dr. Bonura), Georgetown University, Washington, DC; Inova Fairfax Hospital (Drs. Nathan, Ahmad, Shlobin, and S.D. Barnett), Falls Church, VA; and the Division of Pulmonary and Critical Care Medicine (Drs. Osei, Zaiman, Hassoun, Moller, and Girgis), Johns Hopkins University, Baltimore, MD.

Correspondence to: Christopher F. Barnett, MD, MPH, 200 W Arbor Dr, MPF 360, San Diego, CA 92103; e-mail: cbarnett@ucsd.edu


This research was supported in part by the Intramural Research Program of the National Institutes of Health; the National Heart, Lung, and Blood Institute; and Clinical Center, Critical Care Medicine Department.

Dr. Nathan has received lecture/consultant fees from Gilead, United Therapeutics, and Actelion. Dr. Ahmad has received consultant fees from Actelion. Dr. Zaiman has received clinical research support from Actelion. Dr. Hassoun has received clinical research support from Actelion, Gilead, Pfizer, and United Therapeutics. Dr. Girgis has received clinical research support from Pfizer, United Therapeutics, and Lilly/ICOS; and lecture/consultant fees from Gilead and Actelion. Drs. C.F. Barnett, Bonura, Shlobin, Osei, Moller, and S.D. Barnett have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).

For editorial comment see page 1410


© 2009 American College of Chest Physicians


Chest. 2009;135(6):1455-1461. doi:10.1378/chest.08-1881
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Background:  Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined.

Methods:  We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed.

Results:  Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n = 18) increased by 59 m (p = 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 ± 4.3 to 39.4 ± 2.8 mm Hg (p = 0.008). The 1- and 3-year transplant-free survival rates were 90% and 74%, respectively.

Conclusions:  PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.

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