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 analyzing 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 six-month follow-up data were examined to assess clinical progression and predict one-year mortality. Variables included forced vital capacity (FVC), diffusing capacity for carbon monoxide (DL CO ), six-minute 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 ILD cohort of the CARE-PF registry. Results Multivariable analysis identified FVC, DL CO , 6MWD, and BMI as independent predictors of one-year survival. We established three risk groups based on these variables: low-risk (<15% one-year mortality), intermediate-risk (15–30%), and high-risk (>30%). Potential treatment goals were defined based on FVC, DL CO , 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.