Please use this identifier to cite or link to this item:
Title: Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: a systematic review.
Austin Authors: Khor, Yet H ;Ng, Yvonne;Barnes, Hayley;Goh, Nicole S L ;McDonald, Christine F ;Holland, Anne E 
Affiliation: Respiratory and Sleep Medicine
Institute for Breathing and Sleep
Dept of Respiratory Medicine, Alfred Health, Melbourne, Australia
School of Medicine, University of Melbourne, Melbourne, Australia
Dept of Respiratory Medicine, Alfred Health, Melbourne, Australia
Dept of Physiotherapy, Alfred Health and Monash University, Melbourne, Australia
Dept of Respiratory Medicine, Alfred Health, Melbourne, Australia
Monash Lung and Sleep, Monash Health, Clayton, Australia
Issue Date: 30-Sep-2020
Date: 2020-08-04
Publication information: European Respiratory Review 2020; 29(157): 190158
Abstract: In addition to facilitating healthcare delivery planning, reliable information about prognosis is essential for treatment decisions in patients with idiopathic pulmonary fibrosis (IPF). This review aimed to evaluate the prognosis of patients with IPF without anti-fibrotic therapy. We included all cohort studies and the placebo arms of randomised controlled trials (RCTs) in IPF and follow-up of ≥12 months. Two reviewers independently evaluated studies for inclusion, assessed risk of bias and extracted data. A total of 154 cohort studies and 16 RCTs were included. The pooled proportions of mortality were 0.12 (95% CI 0.09-0.14) at 1-2 years, 0.38 (95% CI 0.34-0.42) between 2-5 years, and 0.69 (95% CI 0.59-0.78) at ≥5 years. The pooled mean overall survival was 4 years (95% CI 3.7-4.6) for studies with a follow-up duration of 10 years. At <2 years, forced vital capacity and diffusing capacity of the lung for carbon monoxide declined by a mean of 6.76% predicted (95% CI -8.92 -4.61) and 3% predicted (95% CI -5.14 -1.52), respectively. Although heterogeneity was high, subgroup analyses revealed lower pooled proportions of mortality at 1 year in the RCT participants (0.07 (95% CI 0.05-0.09)) versus cohort study participants (0.14 (95% CI 0.12-0.17)). This review provides comprehensive information on the prognosis of IPF, which can inform treatment discussions with patients and comparisons for future studies with new therapies.
DOI: 10.1183/16000617.0158-2019
ORCID: 0000-0002-5434-9342
Journal: European Respiratory Review
PubMed URL: 32759374
Type: Journal Article
Appears in Collections:Journal articles

Show full item record

Page view(s)

checked on Sep 27, 2023

Google ScholarTM


Items in AHRO are protected by copyright, with all rights reserved, unless otherwise indicated.