Assessing the Therapeutic Response to Pirfenidone in Idiopathic Pulmonary Fibrosis: Can We Do Better than with Forced Vital Capacity Alone?

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

Lung

journal_title

Lung

authors

Hosein K,Le J,Mura M

doi

10.1007/s00408-016-9963-3

subject

Has Abstract

pub_date

2017-02-01 00:00:00

pages

101-105

issue

1

eissn

0341-2040

issn

1432-1750

pii

10.1007/s00408-016-9963-3

journal_volume

195

pub_type

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