RT Journal Article
SR Electronic
T1 The clinical efficacy of pulmonary hypertension-specific agents in idiopathic pulmonary fibrosis: A systematic review and meta-analysis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA1341
DO 10.1183/13993003.congress-2019.PA1341
VO 54
IS suppl 63
A1 Kim, Mi-Ok
A1 Jwa, Hyeyoung
YR 2019
UL http://erj.ersjournals.com/content/54/suppl_63/PA1341.abstract
AB Background: Pulmonary hypertension (PH) is common in patients with idiopathic pulmonary fibrosis (IPF) and is associated with poor outcomes. This study was performed to determine the clinical efficacy of PH-specific therapeutic agents for IPF patients.Methods: We performed a systematic review and meta-analysis using MEDLINE, EMBASE, and the Cochrane Central Register. We searched RCTs without language restriction until November 2018. The primary outcome was all-cause mortality.Results: We analyzed 10 RCTs involving 2,124 patients, 1,274 of whom received PH-specific agents. In pooled estimates, the use of PH-specific agents was not significantly associated with reduced all-cause mortality compared with controls (hazard ratio = 0.99; 95% confidence interval (CI): 0.92–1.06; p = 0.71; I2 = 30%). When we performed subgroup analysis according to type of PH-specific agent, mean FVC ≥60% or <60%, or mean DLco ≥30% or <30%, there was also no significant reduction in all-cause mortality associated with PH-specific agents. We observed a small but significant improvement in quality of life associated with the PH-specific agents group, according to the St. George Respiratory Questionnaire total score (mean difference = -3.16 points; 95% CI: -5.34–-0.97; p = 0.005; I2 = 0%). We found no significant changes from baseline of lung function, dyspnea, and exercise capacity. Serious adverse events were similar between the two groups.Conclusion: Although PH-specific agents provided small health-related quality-of-life benefits, there was insufficient evidence from our meta-analysis to support the use of PH-specific agents in IPF patients.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA1341.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).