Risk stratification as a guide to goal-oriented management of patients with fibrotic interstitial lung disease: a registry-based analysis

Introduction: Idiopathic pulmonary fibrosis (IPF) has high morbidity and mortality with limited treatment options. Goal-oriented management approaches, such as the "treatable traits" concept, have yet to be implemented in IPF. This study aims to identify specific treatment goals in IPF for potential interventions by analysing outcomes from two national registries. Methods: We used data from the INSIGHTS-IPF registry, comprising 1232 IPF patients, enrolled from 2014 to 2020 as derivation cohort. Baseline and 6-month follow-up data were examined to assess clinical progression and predict 1-year mortality. Variables included forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (D _LCO), 6-min walk distance (6MWD), body mass index (BMI) and comorbidities. For validation we used data from 490 IPF patients enrolled in the Canadian Registry for Pulmonary Fibrosis (CARE-PF) and the full 2576 fibrotic interstitial lung disease (ILD) cohort of the CARE-PF registry. Results: Multivariable analysis identified FVC, D _LCO, 6MWD and BMI as independent predictors of 1-year survival. We established three risk groups based on these variables: low risk (<15% 1-year mortality), intermediate risk (15-30%) and high risk (>30%). Potential treatment goals were defined based on FVC, D _LCO, 6MWD and BMI, which are readily available and may be responsive to interventions. Our risk model showed equivalent accuracy in the validation cohort, both for IPF alone and the overall population. Conclusion: This study provides a novel risk model for IPF patients, which may also apply to a broader spectrum of fibrotic ILD. It is based on potentially actionable variables which deserve further evaluation as measurable treatment goals in interventional clinical trials.