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Exacerbation of idiopathic pulmonary fibrosis and connective tissue disease-related interstitial lung disease outcomes: a systematic review and meta-analysis
Journal article   Open access

Exacerbation of idiopathic pulmonary fibrosis and connective tissue disease-related interstitial lung disease outcomes: a systematic review and meta-analysis

Kamonpun Ussavarungsi, Samapon Duangkham, Poemlarp Mekraksakit, Narut Prasitlumkum, Jakrin Kewcharoen, Christopher A Childs and Kenneth Nugent
Future Rare Diseases, Vol.5(1), 2571386
12/31/2025
DOI: 10.1080/23995270.2025.2571386
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https://doi.org/10.1080/23995270.2025.2571386View
Published (Version of record) Open Access

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Abstract

Introduction Acute exacerbations (AE) of idiopathic pulmonary fibrosis (IPF) and connective tissue disease-related interstitial lung disease (CTD-ILD) lack standardized treatments, and outcomes based on ILD etiology remain unclear. Whether adding immunosuppressive drugs to corticosteroids in IPF provides similar benefits as in CTD-ILD remains unknown. This study evaluates mortality and medical therapy effects on AE-IPF and AE-CTD-ILD outcomes. Methods A systematic search was conducted on August 21, 2020, across the Embase, PubMed, and Cochrane databases. Pooled incidence of mortality, mechanical ventilator use, and noninvasive ventilation use in AE-IPF and AE-CTD-ILD was calculated. A random effects model compared different treatment regimens. Results This meta-analysis included 28 studies with 3,148 patients, 1,253 patients had AE (983 IPF and 270 CTD-ILD). The 30-day and 90-day mortality rates for AE-IPF were 36% and 57%, respectively, and for AE-CTD-ILD were 34% and 44%. AE-IPF treated with corticosteroids alone had a 90-day mortality of 63%, while AE-CTD-ILD had 17%. In the corticosteroid with immunosuppressive therapy subgroup, 90-day mortality was 44% for AE-IPF and 61% for AE-CTD-ILD. Conclusion AEs have a poor prognosis regardless of etiology, with no compelling evidence for a definitive treatment regimen. Addressing these uncertainties is crucial for improving outcomes and optimizing care for AE-ILD patients.

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