RT Journal Article
SR Electronic
T1 Epidemiology of interstitial lung diseases and their progressive-fibrosing behaviour in six European countries
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP 00597-2021
DO 10.1183/23120541.00597-2021
VO 8
IS 1
A1 Ole Hilberg
A1 Anna-Maria Hoffmann-Vold
A1 Vanessa Smith
A1 Demosthenes Bouros
A1 Maritta Kilpeläinen
A1 Julien Guiot
A1 Antonio Morais
A1 Susana Clemente
A1 Zoe Daniil
A1 Despina Papakosta
A1 Havard Fretheim
A1 Sofia Neves
A1 Tiago M. Alfaro
A1 Katerina M. Antoniou
A1 Neus Valveny
A1 Guus Asijee
A1 Stéphane Soulard
A1 Wim Wuyts
A1 the PERSEIDS study group
A1 Guy Brusselle
A1 Vassilios Tzilas
A1 Argyris Tzouvelekis
A1 Evangelos Bouros
A1 Katariina Perkonoja
A1 Monique Henket
A1 Marie-Pierre Cunin
A1 Raquel Rosa
A1 Teresa Martín
A1 Joana Branco
A1 Maria Alvarenga
A1 Ilias Dimeas
A1 Theodora Simopoulou
A1 Evangelia Fouka
A1 Ioanna Tsiouprou
A1 Athanasios Zaharias
A1 Sergio Campainha
A1 Carla Nogueira
A1 Sara Freitas
A1 Vânia Fernandes
A1 Carolina Cabo
A1 Irini Vasarmidi
YR 2022
UL http://openres.ersjournals.com/content/8/1/00597-2021.abstract
AB 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.The PERSEIDS study provides updated and detailed epidemiological data for ILDs across six European countries. Prevalences ranged between 26.7–236.8 (F-ILDs), 2.8–31.0 (IPF), 22.3–205.8 (non-IPF F-ILDs). A third of non-IPF F-ILD cases showed PF behaviour. https://bit.ly/3wLH7pF