Abstract:
ABSTARCT:New anti-fibrotic agents for idiopathic pulmonary fibrosis (IPF) were approved based on the results of forced vital capacity (FVC) trends, although concerns were raised about the reliability of FVC as the only endpoint parameter. We hypothesized that IPF-specific multi-dimensional scores (Composite Physiologic Index-CPI; gender-age-physiology-GAP; risk stratification score-RISE) would better capture response to therapy. In this pilot study, treated and untreated cohorts of IPF patients, matched for demographic and functional characteristics were prospectively followed for 1 year, at 4-month intervals. Progression-free survival was significantly improved in treated patients (p = 0.0093). While no difference in FVC longitudinal trends was observed, MRC dyspnea score (p = 0.0347), diffusing lung capacity (p = 0.0015), 6-min walk distance (p = 0.0007), CPI (p = 0.0457) and RISE (p = 0.0005) were significantly stabilized in treated patients, compared to steady worsening in untreated subjects. Multi-dimensional scores provide broader spectrum of prognostic information and may facilitate the assessment of efficacy of new drugs for IPF.
journal_name
Lungjournal_title
Lungauthors
Hosein K,Le J,Mura Mdoi
10.1007/s00408-016-9963-3subject
Has Abstractpub_date
2017-02-01 00:00:00pages
101-105issue
1eissn
0341-2040issn
1432-1750pii
10.1007/s00408-016-9963-3journal_volume
195pub_type
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