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

Ole Hilberg; Anna-Maria Hoffmann-Vold; Vanessa Smith; Demosthenes Bouros; Maritta Kilpeläinen; Julien Guiot; Antonio Morais; Susana Clemente; Zoe Daniil; Despina Papakosta; Havard Fretheim; Sofia Neves; Tiago M Alfaro; Katerina M Antoniou; Neus Valveny; Guus Asijee; Stéphane Soulard; Wim Wuyts

doi:10.1183/23120541.00597-2021

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

ERJ Open Res. 2022 Jan 24;8(1):00597-2021. doi: 10.1183/23120541.00597-2021. eCollection 2022 Jan.

Authors

Ole Hilberg¹, Anna-Maria Hoffmann-Vold², Vanessa Smith³, Demosthenes Bouros^{4

5}, Maritta Kilpeläinen⁶, Julien Guiot⁷, Antonio Morais⁸, Susana Clemente⁹, Zoe Daniil¹⁰, Despina Papakosta¹¹, Havard Fretheim², Sofia Neves¹², Tiago M Alfaro¹³, Katerina M Antoniou¹⁴, Neus Valveny¹⁵, Guus Asijee¹⁶, Stéphane Soulard¹⁶, Wim Wuyts¹⁷

Affiliations

¹ IRS-centre, Lillebælt Hospital, Vejle, Denmark.
² Dept of Rheumatology, Oslo University Hospital - Rikshospitalet, Oslo, Norway.
³ Dept of Rheumatology, Ghent University Hospital, Ghent, Belgium.
⁴ Dept of Pneumonology, Athens Medical Centre, Maroussi, Greece.
⁵ National and Kapodistrian University of Athens, Athens, Greece.
⁶ Dept of Pulmonary Diseases, Turku University Hospital and University of Turku, Turku, Finland.
⁷ Dept of Respiratory Medicine, Liege University Hospital Centre, Liege, Belgium.
⁸ Pulmonary Dept, Sao Joao University Hospital Centre, Porto, Portugal.
⁹ Pulmonary Dept, Beatriz Angelo Hospital, Loures, Portugal.
¹⁰ Dept of Respiratory Medicine, University Hospital of Larissa, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece.
¹¹ Dept of Respiratory Medicine, Aristotle University of Thessaloniki, George Papanikolaou General Hospital, Thessaloniki, Greece.
¹² Pulmonary Dept, Vila Nova de Gaia/Espinho Hospital Centre, Vila Nova de Gaia, Portugal.
¹³ Pulmonary Dept, Centro Hospitalar e Universitario de Coimbra, Coimbra, Portugal.
¹⁴ Dept of Respiratory Medicine, Faculty of Medicine, University of Crete, Crete, Greece.
¹⁵ Trial Form Support S.L., Barcelona, Spain.
¹⁶ Boehringer Ingelheim B.V., Amsterdam, The Netherlands.
¹⁷ Dept of Respiratory Medicine, Leuven University Hospital, Leuven, Belgium.

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 and 83.6 (ILDs), 7.7 and 76.2 (F-ILDs), 0.4 and 10.3 (IPF), 6.6 and 71.7 (non-IPF F-ILDs), and 0.3 and 1.5 (SSc-ILD); and prevalence/10⁵ persons ranged between 33.6 and 247.4 (ILDs), 26.7 and 236.8 (F-ILDs), 2.8 and 31.0 (IPF), 22.3 and 205.8 (non-IPF F-ILDs), and 1.4 and 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 and 14.5/10⁵ person-years, and prevalence between 6.9 and 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.