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.
Highlights
Systemic sclerosis (SSc) is a rare, heterogeneous, chronic, autoimmune disease characterized by fibrosis of the skin and internal organs [1]
Risk factors associated with mortality in SSc-associated Interstitial lung disease (ILD) (SSc-ILD) are male sex, older age, extent of disease on chest high-resolution computed tomography (HRCT), lower forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide (DLco) and pulmonary hypertension (PH) [9, 10]
Real-world database covering most of the population of France, this study provides valuable insights into the epidemiology, mortality, healthcare resource utilization and costs associated with SSc-ILD
Summary
Systemic sclerosis (SSc) is a rare, heterogeneous, chronic, autoimmune disease characterized by fibrosis of the skin and internal organs [1]. ILD is estimated to affect between 19 and 90% of patients with SSc (depending on the study), and around 40% have clinically significant ILD [3,4,5,6,7]. It is the leading cause of death in patients with SSc [2], with a 4.6-fold increased risk of mortality compared with the general population [8]. PH is a common complication in patients with SSc and SSc-ILD, and causes up to 33% of SScrelated deaths [11,12,13]
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