Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)

Mouhamad Nasser,Eric Hachulla,Loic Boussel,Katell Le Lay,Vincent Cottin,Fabienne Bazin,Delphine Maucort-Boulch,Lara Chalabreysse,Sébastien Marque,Jacques Massol,Stéphane Jouneau,Salim Si-Mohamed,Sophie Larrieu,Françoise Thivolet-Bejui

doi:10.1186/s12931-021-01749-1

Abstract

BackgroundThere is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-ILD) in France. An algorithm for extracting claims data was developed to indirectly identify and describe patients with PF-ILD in the French national administrative healthcare database.MethodsThe French healthcare database, the Système National des Données de Santé (SNDS), includes data related to ambulatory care, hospitalisations and death for 98.8% of the population. In this study, algorithms based on age, diagnosis and healthcare consumption were created to identify adult patients with PF-ILD other than idiopathic pulmonary fibrosis between 2010 and 2017. Incidence, prevalence, survival estimates, clinical features and healthcare resource usage and costs were described among patients with PF-ILD.ResultsWe identified a total of 14,413 patients with PF-ILD. Almost half of them (48.1%) were female and the mean (± standard deviation) age was 68.4 (± 15.0) years. Between 2010 and 2017, the estimated incidence of PF-ILD ranged from 4.0 to 4.7/100,000 person-years and the estimated prevalence from 6.6 to 19.4/100,000 persons. The main diagnostic categories represented were exposure-related ILD other than hypersensitivity pneumonitis (n = 3486; 24.2%), idiopathic interstitial pneumonia (n = 3113; 21.6%) and rheumatoid arthritis-associated ILD (n = 2521; 17.5%). Median overall survival using Kaplan–Meier estimation was 3.7 years from the start of progression. During the study, 95.2% of patients had ≥ 1 hospitalisation for respiratory care and 34.3% were hospitalised in an intensive care unit. The median (interquartile range) total specific cost per patient during the follow-up period was €25,613 (10,622–54,287) and the median annual cost per patient was €18,362 (6856–52,026), of which €11,784 (3003–42,097) was related to hospitalisations. Limitations included the retrospective design and identification of cases through an algorithm in the absence of chest high-resolution computed tomography scans and pulmonary function tests.ConclusionsThis large, real-world, longitudinal study provides important insights into the characteristics, epidemiology and healthcare resource utilisation and costs associated with PF-ILD in France using a comprehensive and exhaustive database, and provides vital evidence that PF-ILD represents a high burden on both patients and healthcare services.Trial registration ClinicalTrials.gov, NCT03858842. ISRCTN, ISRCTN12345678. Registered 3 January 2019—Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03858842

Highlights

There is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-Interstitial lung disease (ILD)) in France
Nasser et al Respir Res (2021) 22:162. This large, real-world, longitudinal study provides important insights into the characteristics, epide‐ miology and healthcare resource utilisation and costs associated with progressive fibrosing interstitial lung disease (PF-ILD) in France using a comprehensive and exhaustive database, and provides vital evidence that PF-ILD represents a high burden on both patients and health‐ care services
While the study design limited the conclusions that could be drawn from the data, in this study, pirfenidone was observed to decrease the rate of decline in lung function measured in-clinic, indicating that it may benefit patients with progressive fibrosing unclassifiable ILD

Summary

Introduction

There is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-ILD) in France. In some patients with fibrosing ILDs, a progressive fibrosing phenotype develops comparable to that observed in idiopathic pulmonary fibrosis (IPF), including worsening respiratory symptoms, decline in lung function and early mortality despite standard of care treatment [2, 4, 5]. While the study design limited the conclusions that could be drawn from the data, in this study, pirfenidone was observed to decrease the rate of decline in lung function measured in-clinic (a secondary endpoint), indicating that it may benefit patients with progressive fibrosing unclassifiable ILD

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