Document Detail

Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience.
MedLine Citation:
PMID:  19118270     Owner:  NLM     Status:  MEDLINE    
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.
Christopher F Barnett; Eric J Bonura; Steven D Nathan; Shahzad Ahmad; Oksana A Shlobin; Kwabena Osei; Ari L Zaiman; Paul M Hassoun; David R Moller; Scott D Barnett; Reda E Girgis
Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, N.I.H., Intramural     Date:  2008-12-31
Journal Detail:
Title:  Chest     Volume:  135     ISSN:  1931-3543     ISO Abbreviation:  Chest     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-06-05     Completed Date:  2009-07-07     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  0231335     Medline TA:  Chest     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1455-61     Citation Subset:  AIM; IM    
Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD, USA.
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MeSH Terms
Administration, Inhalation
Administration, Oral
Antihypertensive Agents / administration & dosage*
Cohort Studies
Drug Therapy, Combination
Epoprostenol / administration & dosage
Follow-Up Studies
Hemodynamics / drug effects*,  physiology
Hypertension, Pulmonary / drug therapy*,  etiology*,  mortality
Iloprost / administration & dosage
Kaplan-Meier Estimate
Linear Models
Middle Aged
Piperazines / administration & dosage
Purines / administration & dosage
Respiratory Function Tests
Retrospective Studies
Risk Assessment
Sarcoidosis / complications*,  diagnosis,  mortality
Severity of Illness Index
Statistics, Nonparametric
Sulfonamides / administration & dosage
Sulfones / administration & dosage
Survival Rate
Treatment Outcome
Reg. No./Substance:
0/Antihypertensive Agents; 0/Piperazines; 0/Purines; 0/Sulfonamides; 0/Sulfones; 35121-78-9/Epoprostenol; 3M7OB98Y7H/sildenafil; 78919-13-8/Iloprost; Q326023R30/bosentan
Comment In:
Chest. 2009 Jun;135(6):1410-2   [PMID:  19497891 ]

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