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Meta-Analysis
. 2020 Aug 4;29(157):190158.
doi: 10.1183/16000617.0158-2019. Print 2020 Sep 30.

Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: a systematic review

Yet H Khor  1   2   3   4 , Yvonne Ng  5 , Hayley Barnes  4 , Nicole S L Goh  6   2   3   4 , Christine F McDonald  6   2   3 , Anne E Holland  2   7
Affiliations
Meta-Analysis

Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: a systematic review

Yet H Khor et al. Eur Respir Rev. .

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.

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Conflict of interest statement

Conflict of interest: Y.H. Khor reports grants from National Health and Medical Research Council Postgraduate Scholarship, during the conduct of the study; other from Air Liquide Healthcare, grants and personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work. Conflict of interest: Y. Ng has nothing to disclose. Conflict of interest: H. Barnes has nothing to disclose. Conflict of interest: N.S.L. Goh reports grants and personal fees from Boehringer Ingelheim, non-financial support from Air liquide, personal fees from Roche, outside the submitted work. Conflict of interest: C.F. McDonald reports non-financial support from Air Liquide Healthcare, other from AstraZeneca and Menarini, grants from Boehringer Ingelheim, outside the submitted work. Conflict of interest: A.E. Holland reports grants from Roche, personal fees from Boehringer Ingelheim, non-financial support from BOC Healthcare and Air Liquide, outside the submitted work.

Figures

FIGURE 1
FIGURE 1
Study flow diagram. RCTs: randomised controlled trials.
FIGURE 2
FIGURE 2
Pooled proportions of mortality at 1 year to <2 years. ES: effect size.
FIGURE 3
FIGURE 3
Pooled proportions of mortality between 2–5 years. ES: effect size.
FIGURE 4
FIGURE 4
Pooled proportions of mortality at ≥5 years. ES: effect size.
FIGURE 5
FIGURE 5
Pooled mean changes in forced vital capacity (FVC) at 1 year to <2 years.
FIGURE 6
FIGURE 6
Pooled mean changes in diffusing capacity of the lung for carbon monoxide (DLCO) at 1 year to <2 years.
See this image and copyright information in PMC

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