Treatment of sarcoidosis-associated pulmonary hypertension: A two-center experience

Christopher F. Barnett, Eric J. Bonura, Steven D. Nathan, Shahzad Ahmad, Oksana A. Shlobin, Kwabena Osei, Ari L. Zaiman, Paul M Hassoun, David Moller, Scott D. Barnett, Reda E. Girgis

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1455-1461
Number of pages7
JournalChest
Volume135
Issue number6
DOIs
StatePublished - Jun 1 2009

Fingerprint

Sarcoidosis
Pulmonary Hypertension
Hemodynamics
Exercise
Therapeutics
Transplants
Mortality
Respiratory Function Tests
Pulmonary Artery
Referral and Consultation
Survival Rate
Retrospective Studies
Pressure
Lung
Survival
Population

Keywords

  • Bosentan
  • Prostacyclin
  • Pulmonary hypertension
  • Sarcoidosis
  • Sildenafil

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Barnett, C. F., Bonura, E. J., Nathan, S. D., Ahmad, S., Shlobin, O. A., Osei, K., ... Girgis, R. E. (2009). Treatment of sarcoidosis-associated pulmonary hypertension: A two-center experience. Chest, 135(6), 1455-1461. https://doi.org/10.1378/chest.08-1881

Treatment of sarcoidosis-associated pulmonary hypertension : A two-center experience. / Barnett, Christopher F.; Bonura, Eric J.; Nathan, Steven D.; Ahmad, Shahzad; Shlobin, Oksana A.; Osei, Kwabena; Zaiman, Ari L.; Hassoun, Paul M; Moller, David; Barnett, Scott D.; Girgis, Reda E.

In: Chest, Vol. 135, No. 6, 01.06.2009, p. 1455-1461.

Research output: Contribution to journalArticle

Barnett, CF, Bonura, EJ, Nathan, SD, Ahmad, S, Shlobin, OA, Osei, K, Zaiman, AL, Hassoun, PM, Moller, D, Barnett, SD & Girgis, RE 2009, 'Treatment of sarcoidosis-associated pulmonary hypertension: A two-center experience', Chest, vol. 135, no. 6, pp. 1455-1461. https://doi.org/10.1378/chest.08-1881
Barnett CF, Bonura EJ, Nathan SD, Ahmad S, Shlobin OA, Osei K et al. Treatment of sarcoidosis-associated pulmonary hypertension: A two-center experience. Chest. 2009 Jun 1;135(6):1455-1461. https://doi.org/10.1378/chest.08-1881
Barnett, Christopher F. ; Bonura, Eric J. ; Nathan, Steven D. ; Ahmad, Shahzad ; Shlobin, Oksana A. ; Osei, Kwabena ; Zaiman, Ari L. ; Hassoun, Paul M ; Moller, David ; Barnett, Scott D. ; Girgis, Reda E. / Treatment of sarcoidosis-associated pulmonary hypertension : A two-center experience. In: Chest. 2009 ; Vol. 135, No. 6. pp. 1455-1461.
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abstract = "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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AU - Shlobin, Oksana A.

AU - Osei, Kwabena

AU - Zaiman, Ari L.

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AU - Girgis, Reda E.

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