Occurrence of Idiopathic Pulmonary Fibrosis in Italy: Latest Evidence from Real-World Data.

Federico Mei,Andrea Faragalli,Martina Bonifazi,Lara Letizia Latini,Marica Iommi,Luigi Ferrante,Rosaria Gesuita,Flavia Carle,Edlira Skrami,Liana Spazzafumo

doi:10.3390/ijerph19052510

Abstract

The aim of the study was to evaluate the trend in the incidence of idiopathic pulmonary fibrosis (IPF) in a real-world setting of the Marche region, a region of Central Italy, between 2014 and 2019. This observational prospective study was based on administrative databases of hospital discharges and drug prescriptions. All adult residents in the Marche Region with a first prescription of antifibrotic drugs, or a first hospitalization with a diagnosis of IPF during the study period, were identified as incident cases of IPF. A multiple Poisson regression analysis was used to estimate the IPF incidence trend, adjusted for age, sex, and health conditions. The mean incidence rate was 9.8 cases per 100,000 person-years. A significant increasing trend of 6% per year was observed. The incidence rates were significantly higher in males than females, older subjects, and those with poorer health conditions. To our knowledge, this is the first study evaluating incidences of IPF over a 6-year period in Italy, combining hospital discharge and drug prescription databases. The study highlights that the combined use of two secondary sources is a reliable strategy to accurately identify new cases of IPF when the appropriate disease registry is lacking.

Highlights

Idiopathic pulmonary fibrosis (IPF) is a rare, chronic, and progressive disease, causing an irreversible decline of lung function over time that dramatically impairs the quality of life
Considering the Broad Case Definition (BCD) algorithm, the true positive rate dropped to 64.8% (92 out of 142), and with the Narrow Case Definition (NCD) algorithm decreased to 58.5% (83 out of 142)
Between 2014 and 2019, 1037 prevalent and 766 new IPF cases were found using the General Case Definition (GCD) algorithm, with an overall prevalence of 13.3 per 100,000 residents and a mean incidence rate of 9.8 per 100,000 py

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a rare, chronic, and progressive disease, causing an irreversible decline of lung function over time that dramatically impairs the quality of life. It mainly occurs in older male people, and it is characterized by a poor prognosis, with a median survival time ranging between 3 and 5 years [1]. Epidemiological estimates showed huge geographical variability [1,4], with the highest incidence rates in North America and East Europe 100,000 person-years) and the lowest in East Asia Reasons for worldwide variability in IPF incidence and prevalence include potential differences in genetics and risk factor exposure among countries, as well as methodological IPF incidence is increasing worldwide [5], as well as hospitalization [6] and mortality [7], indicating an increasing burden of the disease on the population and health systems.

Objectives

Methods

Results

Conclusion