Potential benefit of bosentan therapy in borderline or less severe pulmonary hypertension secondary to idiopathic pulmonary fibrosis—an interim analysis of results from a prospective, single-center, randomized, parallel-group study

BMC Pulmonary Medicine

Research Article

Akihiko Gemma¹  Mitsunori Hino²  Yosuke Tanaka² 

[1] Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, 113-8603, Tokyo, Japan;Department of Respiratory Medicine, Nippon Medical School, Chiba Hokusoh Hospital, 1715 Kamagari, 270-1694, Inzai, Chiba, Japan;

关键词: Pulmonary hypertension;    Idiopathic pulmonary fibrosis;    Right heart catheterization;    Echocardiography;    Endothelin receptor antagonists;   

DOI  :  10.1186/s12890-017-0523-2

 received in 2017-03-27, accepted in 2017-11-24,  发布年份 2017

来源: Springer

【 摘 要 】

BackgroundNo drugs have been approved for the treatment of patients with pulmonary hypertension (PH) secondary to idiopathic pulmonary fibrosis (IPF), particularly those with idiopathic honeycomb lung. This study was conducted to investigate the long-term efficacy and safety of bosentan for PH based on changes in prognosis and respiratory failure.MethodsIPF patients with borderline or less severe PH and completely organized honeycomb lung were randomized (1:1) to bosentan or no treatment for PH for 2 years and assessed at baseline and every 6 months for respiratory failure, activities of daily living (ADL), lung and heart functions by right cardiac catheterization, and other parameters. An interim analysis was performed, however, following detection of a significant survival benefit favoring bosentan therapy.ResultsSignificant differences were noted for the bosentan-treated (n = 12) vs. untreated (n = 12) groups in hospital-free survival (603.44 ± 50.074 days vs. 358.87 ± 68.65 days; hazard ratio [HR], 0.19; P = 0.017) and overall survival (671 days vs. 433.78 ± 66.98 days; HR, 0.10; P = 0.0082). Again, significant improvements were noted for the bosentan-treated group from baseline to month 6 or 12 in several indices in ADL, pulmonary circulation, and %DLCO. Without requiring O2 inhalation, bosentan was associated with no increase but a trend toward a decrease in adverse events and an improvement in respiratory status.ConclusionsBosentan tended to improve prognosis and ADL without worsening respiratory failure in IPF patients with borderline or less severe PH and completely organized honeycomb lung alone.Trial registrationThis study was registered on December 18, 2010 with UMIN-CTR Clinical Trial as UMIN000004749 to investigate the long-term influence of bosentan on cardiac function, as well as its cardioprotective efficacy and safety, in patients with pulmonary hypertension secondary to concurrent COPD and IPF, respectively.

【 授权许可】

CC BY   
© The Author(s). 2017