Epidemiology of interstitial lung diseases and their progressive-fibrosing behaviour in six European countries

Abstract

The PERSEIDS study aimed to estimate incidence/prevalence of interstitial lung diseases (ILDs), fibrosing Interstitial lung diseases (F-ILDs), idiopathic pulmonary fibrosis (IPF), systemic sclerosis-associated ILD (SSc-ILD), other non-IPF F-ILDs and their progressive-fibrosing (PF) forms in six European countries, as current data are scarce.This retrospective, two-phase study used aggregate data (2014–2018). In Phase 1, incident/prevalent cases of ILDs above were identified from clinical databases through an algorithm based on codes/keywords, and incidence/prevalence was estimated. For non-IPF F–ILDs, the relative percentage of subtypes was also determined. In Phase 2, a subset of non-IPF F-ILD cases was manually reviewed to determine the percentage of PF behaviour and usual interstitial pneumonia-like (UIP-like) pattern. A weighted mean percentage of progression was calculated for each country and used to extrapolate incidence/prevalence of progressive-fibrosing ILDs (PF–ILDs).In 2018, incidence/105 person-years ranged between 9.4–83.6(ILDs), 7.7–76.2(F-ILDs), 0.4–10.3(IPF), 6.6–71.7(non-IPF F-ILDs) and 0.3–1.5(SSc-ILD); and prevalence/105 persons ranged between 33.6–247.4(ILDs), 26.7–236.8(F-ILDs), 2.8–31.0(IPF), 22.3–205.8(non-IPF F-ILDs) and 1.4–10.1(SSc-ILD). Among non-IPF F-ILDs, sarcoidosis was the most frequent subtype. PF behaviour and UIP-like pattern were present in a third of non-IPF F-ILD cases each and hypersensitivity pneumonitis showed the highest percentage of progressive behaviour. Incidence of PF-ILDs ranged between 2.1–14.5/105 person-years, and prevalence between 6.9–78.0/105 persons.To our knowledge, PERSEIDS is the first study assessing incidence, prevalence and rate of progression of ILDs across several European countries. Still below the threshold for orphan diseases, the estimates obtained were higher and more variable than reported in previous studies, but differences in study design/population must be considered.

Epidemiology and real-life experience in progressive pulmonary fibrosis

“…Hypersensitivity pneumonitis showed the highest percentage of progressive behaviour. Incidence of PF-ILDs ranged between 2.1 and 14.5/10 5 person-years, and prevalence between 6.9 and 78.0/10 5 persons [19].…”

Progressive fibrosing interstitial lung disease in rheumatoid arthritis: A retrospective study

“…Nearly 50% of RA-ILD patients showed a PF-ILD phenotype in the present study. This percentage is higher than in the PERSEID study, a large European retrospective study, demonstrating a progressive pattern in 38% of RA-ILD patients (13). We believe that screening PF-ILD patients is of potential interest for the therapeutic management of RA-ILD patients.…”

“…It typically develops in the fifth or sixth decade and can be diagnosed up to 10 years after RA but sometimes occurs before joint symptoms (4,(9)(10)(11)(12). According to the PERSEIDS study, it's prevalence in Europe ranges from 1 to 18.1 per 10 5 persons and among all subtypes of ILDs that are non-idiopathic pulmonary fibrosis (IPF), RA-ILD had the highest incidence in Belgium in 2018 (13).…”

Epidemiology and comorbidities in idiopathic pulmonary fibrosis: a nationwide cohort study

“…Likewise, because only patients diagnosed with IPF were included in this study, this result could not be applied to patients with progressive pulmonary fibrosis with other ILD subtypes. Because recently published studies suggested operational definition of progressive pulmonary fibrosis with other ILD subtypes, it can be possible to investigate the prevalence of comorbidities in progressive pulmonary fibrosis patients with other ILD subtypes by using nationwide claim data [ 51 , 52 ]. Finally, because this study was a nationwide population-based study based on health claims data, we could only determine the associations between variables, rather than causal relationships.…”