Ambrisentan for sarcoidosis associated pulmonary hypertension

摘要: Background: Sarcoidosis associated pulmonary hypertension (SAPH) is with significant morbidity and mortality. There a paucity of information concerning therapy for this condition. Methods:We performed prospective, open-label, proof concept trial ambrisentan SAPH. 21 subjects SAPH received 5 mg/day 4 weeks then 10/mg day 20 subsequent weeks. Results: No change was noted in the 6-minute walk distance over course study (mean between week 0 24: 9.8 ± 54.6 meters, p: NS). were also no differences 24 terms dyspnea as measured by modified Borg scale, serum brain naturetic peptide, diffusing capacity, quality life Short Form-36. high dropout rate: overall: 11/21, 52%; social reasons: 3/21, 14%; medical 8/21, 38% because dyspnea: 6/21, 29% and/or edema :4/21, 19%. Of those who completed (10/21, 48%), there an improvement theirWHO functional class marked their health related St. George Respiratory questionnaire (-15.3 25.0).However both these improvments did not reach statistical significance possibly small sample size. Conclusion: Although well tolerated many SAPH, remained 24-week trial, improvements WHO suggested possible benefit drug selected patients.