Document Detail

Ambrisentan for sarcoidosis associated pulmonary hypertension.
MedLine Citation:
PMID:  22117505     Owner:  NLM     Status:  In-Process    
BACKGROUND: Sarcoidosis associated pulmonary hypertension (SAPH) is associated with significant morbidity and mortality. There is a paucity of information concerning therapy for this condition.
METHODS: We performed a prospective, open-label, proof of concept trial of ambrisentan for SAPH. 21 subjects with SAPH received 5 mg/day of ambrisentan for 4 weeks and then 10/mg day for 20 subsequent weeks.
RESULTS: No significant change was noted in the 6-minute walk distance over the course of the study (mean change between week 0 and 24: 9.8 +/- 54.6 meters, p: NS). There were also no significant differences between weeks 0 and 24 in terms of dyspnea as measured by the modified Borg scale, serum brain naturetic peptide, diffusing capacity, and quality of life as measured by the Short Form-36. There was a high dropout rate: overall: 11/21, 52%; social reasons: 3/21, 14%; medical reasons: 8/21, 38% because of dyspnea: 6/21, 29% and/or edema: 4/21, 19%. Of those who completed the 24 week study (10/21, 48%), there was an improvement in their WHO functional class and a marked improvement in their health related quality of life as measured by the St. George Respiratory questionnaire (-15.3 +/- 25.0). However both these improvments did not reach statistical significance possibly because of the small sample size.
CONCLUSION: Although ambrisentan was not well tolerated by many of these subjects with SAPH, in those who remained in this 24-week trial, improvements in WHO functional class and in health related quality of life suggested a possible benefit of this drug in selected patients.
M A Judson; K B Highland; S Kwon; J F Donohue; R Aris; N Craft; S Burt; H J Ford
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG / World Association of Sarcoidosis and Other Granulomatous Disorders     Volume:  28     ISSN:  1124-0490     ISO Abbreviation:  Sarcoidosis Vasc Diffuse Lung Dis     Publication Date:  2011 Oct 
Date Detail:
Created Date:  2011-11-28     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9610928     Medline TA:  Sarcoidosis Vasc Diffuse Lung Dis     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  139-45     Citation Subset:  IM    
Division of Pulmonary and Critical Care Medicine, Medical University of South Carolina, USA.
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