Defining and predicting progression in non-IPF interstitial lung disease

Area of Science:

• Pulmonology
• Fibrotic Interstitial Lung Disease
• Clinical Research

Background:

• Antifibrotic treatment shows efficacy in progressive fibrosing interstitial lung disease (fILD) beyond idiopathic pulmonary fibrosis (IPF).
• Lack of consensus on progression definition and scarce real-world data for non-IPF fILD patients hinder treatment strategies.
• Real-world clinical data on non-IPF fILD patients are limited, impacting treatment decisions.

Purpose of the Study:

• To evaluate different definitions of disease progression in non-IPF fILD patients using criteria from major clinical trials.
• To identify predictors of progression and mortality in a real-world cohort of non-IPF fILD patients.
• To assess the prevalence of progressive disease and survival outcomes in non-IPF fILD.

Main Methods:

• Retrospective analysis of 120 non-IPF fILD patients from University Hospitals Leuven (2012-2016).
• Evaluation of disease progression based on INBUILD, RELIEF, and uILD study selection criteria at hospital visits.
• Univariate and multivariate analyses to determine predictors of progression and mortality.

Main Results:

• A high prevalence of progressive disease was observed across different trial definitions (68.3% INBUILD, 54.2% RELIEF, 65.8% uILD).
• Significant overlap in patient identification as progressive fILD across criteria was noted.
• Hypersensitivity pneumonitis (HP) and unclassifiable interstitial lung disease (uILD) were independent predictors of progression, while mortality predictors varied.

Conclusions:

• Non-IPF fILD patients exhibit a high prevalence of progressive disease, but progression is often identified late.
• There is a discrepancy between predictors of mortality and progression in this cohort.
• Development of novel progression predictors is crucial for timely treatment guidance in non-IPF fILD.