Epidemiology, Mortality and Healthcare Resource Utilization Associated With Systemic Sclerosis-Associated Interstitial Lung Disease in France

Abstract

Objectives: To investigate the clinical characteristics, epidemiology, survival estimates and healthcare resource utilization and associated costs in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) in France. Methods: The French national administrative healthcare database, the Système National des Données de Santé (SNDS), includes data on 98.8% of the French population, including data relating to ambulatory care, hospitalizations and death. In our study, claims data from the SNDS were used to identify adult patients with SSc-ILD between 2010 and 2017. We collected data on clinical features, incidence, prevalence, survival estimates, healthcare resource use and costs. Results: In total, 3,333 patients with SSc-ILD were identified, 76% of whom were female. Patients had a mean age [standard deviation (SD)] of 60.6 (14.4) years and a mean (SD) individual study duration of 3.9 (2.7) years. In 2016, the estimated overall incidence and prevalence were 0.69/100,000 individuals and 5.70/100,000 individuals, respectively. The overall survival estimates of patients using Kaplan-Meier estimation were 93, 82, and 55% at 1, 3, and 8 years, respectively. During the study, 98.7% of patients had ≥1 hospitalization and 22.3% of patients were hospitalized in an intensive care unit. The total annual mean healthcare cost per patient with SSc-ILD was €25,753, of which €21,539 was related to hospitalizations. Conclusions: This large, real-world longitudinal study provides important insights into the epidemiology of SSc-ILD in France and shows that the disease is associated with high mortality, healthcare resource utilization and costs. SSc-ILD represents a high burden on both patients and healthcare services. Clinical Trial Registration:www.ClinicalTrials.gov, identifier: NCT03858842.

Keywords: cost; epidemiology; pulmonary fibrosis; scleroderma; systemic sclerosis.

Conflict of interest statement

VC reports personal fees and non-financial support from Actelion, Bayer/MSD, and Promedior/Roche, grants, personal fees, and non-financial support from Boehringer Ingelheim, and personal fees from Novartis, Sanofi, Celgene, Galapagos, and Galecto, outside the submitted work. SL, LB, SS-M, FB, SM, FT-B, and LC have nothing to disclose. JM reports personal fees from Boehringer Ingelheim during the conduct of the study. He reports personal fees from Radico Cohorts (INSERM) for methodologic advice and other fees from Boehringer Ingelheim for methodologic advice and participation in a feasibility study on Praxbind, outside the submitted work. DM-B reports personal fees from MaatParma, outside the submitted work. SJ has received fees, funding or reimbursement for national and international conferences, boards, expert or opinion groups, and research projects over the past 5 years from Actelion, AIRB, AstraZeneca, Bellerophon Therapeutics, Biogen, BMS, Boehringer Ingelheim, Chiesi, Fibrogen, Galecto Biotech, Genzyme, Gilead, GSK, LVL, Mundipharma, Novartis, Olam Pharm, Pfizer, Pliant Therapeutics, Roche, Sanofi and Savara-Serendex. EH reports consulting fees/meeting fees from Actelion, Boehringer Ingelheim, Bayer, GSK, Roche-Chugai, Sanofi-genzyme; speaking fees from Actelion, GSK, Roche-Chugai; and research funding from Octapharma, CSL Behring, GSK, Roche-Chugai and Actelion. JC is an employee of Boehringer Ingelheim France. MN reports non-financial support from Hoffmann-La Roche, Actelion, AstraZeneca and Boehringer Ingelheim outside the submitted work.

Copyright © 2021 Cottin, Larrieu, Boussel, Si-Mohamed, Bazin, Marque, Massol, Thivolet-Bejui, Chalabreysse, Maucort-Boulch, Jouneau, Hachulla, Chollet and Nasser.

Figures

Figure 1

Patient selection. *An eligible adult SSc-ILD patient was defined as a patient with either ≥1 hospital stay with a diagnosis code (principal, related or associated) of lung fibrosis, or ≥1 hospital stay with a diagnosis code (principal, related or associated) of SSc and/or a patient who benefited from full coverage for SSc (patients fully reimbursed for their claims related to SSc). ILD diagnosis could be made after, or within 6 months prior to, SSc diagnosis. ILD, interstitial lung disease; SNDS, Système National des Données de Santé; SSc, systemic sclerosis.

Figure 2

Overall survival estimates for patients with SSc-ILD. (A) Overall survival estimates for all patients, (B) by sex and (C) by age in years. The shading indicates 95% Hall-Wellner band. SSc-ILD, systemic sclerosis-associated interstitial lung disease.