[en] 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.
Hoffmann-Vold, Anna-Maria; Dept of Rheumatology, Oslo University Hospital - Rikshospitalet, Oslo, Norway
Smith, Vanessa ; Dept of Rheumatology, Ghent University Hospital, Ghent, Belgium
Bouros, Demosthenes ; Dept of Pneumonology, Athens Medical Centre, Maroussi, Greece ; National and Kapodistrian University of Athens, Athens, Greece
Kilpeläinen, Maritta; Dept of Pulmonary Diseases, Turku University Hospital and University of Turku, Turku, Finland
GUIOT, Julien ; Centre Hospitalier Universitaire de Liège - CHU > > Service de pneumologie - allergologie
Morais, Antonio ; Pulmonary Dept, Sao Joao University Hospital Centre, Porto, Portugal
Clemente, Susana; Pulmonary Dept, Beatriz Angelo Hospital, Loures, Portugal
Daniil, Zoe; Dept of Respiratory Medicine, University Hospital of Larissa, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece
Papakosta, Despina; Dept of Respiratory Medicine, Aristotle University of Thessaloniki, George Papanikolaou General Hospital, Thessaloniki, Greece
Fretheim, Havard; Dept of Rheumatology, Oslo University Hospital - Rikshospitalet, Oslo, Norway
Neves, Sofia; Pulmonary Dept, Vila Nova de Gaia/Espinho Hospital Centre, Vila Nova de Gaia, Portugal
Alfaro, Tiago M ; Pulmonary Dept, Centro Hospitalar e Universitario de Coimbra, Coimbra, Portugal
Antoniou, Katerina M; Dept of Respiratory Medicine, Faculty of Medicine, University of Crete, Crete, Greece
Valveny, Neus; Trial Form Support S.L., Barcelona, Spain
Asijee, Guus; Boehringer Ingelheim B.V., Amsterdam, The Netherlands
Soulard, Stéphane; Boehringer Ingelheim B.V., Amsterdam, The Netherlands
Wuyts, Wim ; Dept of Respiratory Medicine, Leuven University Hospital, Leuven, Belgium
Support statement: This work was supported by Boehringer Ingelheim International GmbH. Employees were involved in the study design and data analysis, and in the writing of this manuscript. Funding information for this article has been deposited with the Crossref Funder Registry.Conflict of interest: O. Hilberg has nothing to disclose. A-M. Hoffmann-Vold had a consultancy and medical writing relationship with Boehringer Ingelheim; received unrestricted grants from Bayer and Boehringer Ingelheim; received consulting fees from Actelion, Boehringer Ingelheim, ARXX and Medscape; received honoraria for lectures and presentations from Actelion, Boehringer Ingelheim, Roche, Merck Sharp & Dohme, Lilly and Medscape; received support for attending meetings and/or travel from Actelion, Boehringer Ingelheim, Roche and Medscape; and was board member of EUSTAR and Nordic PH group. V. Smith received grants to research support, as senior clinical investigator from Research Foundation Flanders and Boehringer Ingelheim, research grant from Belgian Fund for Scientific Research in Rheumatic Diseases and educational grant from Janssen-Cilag; received consultancy fees from Boehringer Ingelheim; received honoraria for lectures, presentations, and speaker fees from Accord Healthcare, UCB, Boehringer Ingelheim and Janssen-Cilag; received support for attending meetings and/or travel from Celgene and Boehringer Ingelheim; and was chair (unpaid) to EULAR Study group on Microcirculation in Rheumatic Diseases, co-chair (unpaid) to ACR Study Group on Microcirculation and SCTC working group, and steering committee member (unpaid) to ERN-ReCONNET. D. Bouros received consulting fees from Boehringer Ingelheim; received honoraria from Boehringer Ingelheim, Roche and AstraZeneca; received support for attending meetings and/or travel from Boehringer Ingelheim and Roche; and received other financial or nonfinancial interests from Chiesi and ELPEN. M. Kilpelainen has nothing to disclose. J. Guiot has nothing to disclose. A. Morais has nothing to disclose. S. Clemente received payment for lectures and presentations and manuscript writing from Boehringer Ingelheim; and was ad hoc expert member of EMEA ( January 2020). Z. Daniil has nothing to disclose. D. Papakosta has nothing to disclose. H. Fretheim has nothing to disclose. S. Neves has nothing to disclose. T.M. Alfaro received consulting fees from Boehringer Ingelheim and Roche; received payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boehringer Ingelheim and Roche; received support for attending meetings and/or travel from Boehringer Ingelheim and Roche; and participated on a Data Safety Monitoring Board or Advisory Board of Boehringer Ingelheim and Roche. K.M. Antoniou received consulting fees from Boehringer Ingelheim and Roche; received payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boehringer Ingelheim and Roche; received support for attending meetings and/or travel from Boehringer Ingelheim and Roche; and had leadership or fiduciary role in ERS Assembly 12 Secretary (unpaid). N. Valveny received funding to TFS for study conduction and medical writing from Boehringer Ingelheim and is employee of TFS. G. Asijee is employee of Boehringer Ingelheim. S. Soulard is employee of Boehringer Ingelheim. W. Wuyts received grants from Boehringer Ingelheim, Roche and Galapagos.
Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ ALAT clinical practice guideline. Am J Respir Crit Care Med 2018; 198: e44–e68.
Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788–824.
Kreuter M, Swigris J, Pittrow D, et al. The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry. Respir Res 2019; 20: 59.
Antoniou K, Markopoulou K, Tzouvelekis A, et al. Efficacy and safety of nintedanib in a Greek multicentre idiopathic pulmonary fibrosis registry: a retrospective, observational, cohort study. ERJ Open Res 2020; 6: 00172-2019.
Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev 2019; 28: 180100.
Wuyts WA, Papiris S, Manali E, et al. The burden of progressive fibrosing interstitial lung disease: a DELPHI approach. Adv Ther 2020; 37: 3246–3264.
Jaeger VK, Wirz EG, Allanore Y, et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS ONE 2016; 11: e0163894.
Hoffmann-Vold A-M, Fretheim H, Halse A-K, et al. Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med 2019; 200: 1258–1266.
Hoffmann-Vold A-M, Bendstrup E, Dimitroulas T, et al. Identifying unmet needs in SSc-ILD by semi-qualitative in-depth interviews. Rheumatology (Oxford) 2021; 60: 5601–5609.
Olson AL, Gifford AH, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev 2018; 27: 180077.
Olson A, Hartmann N, Patnaik P, et al. Estimation of the prevalence of progressive fibrosing interstitial lung diseases: systematic literature review and data from a physician Survey. Adv Ther 2021; 38: 854–867.
Danish Health Authority (Sundhedsstyrelsen). Researcher machine. 2019. https://sundhedsdatasty relsen.dk/da/forskerservice/forskermaskinen Date last accessed: 3 July 2020.
Auria Clinical Informatics. For researchers. 2020. www.auria.fi/tietopalvelu/en/tutkijalle/index.html Date last accessed: 3 July 2020.
Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018; 27: 180076.
Thomeer M, Demedts M, Vandeurzen K, et al. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin Belg 2001; 56: 163–172.
Karakatsani A, Papakosta D, Rapti A, et al. Epidemiology of interstitial lung diseases in Greece. Respir Med 2009; 103: 1122–1129.
Kornum JB, Christensen S, Grijota M, et al. The incidence of interstitial lung disease 1995-2005: a Danish nationwide population-based study. BMC Pulm Med 2008; 8: 24.
Hyldgaard C, Hilberg O, Muller A, et al. A cohort study of interstitial lung diseases in central Denmark. Respir Med 2014; 108: 793–799.
Bendstrup E, Hyldgaard C, Altraja A, et al. Organisation of diagnosis and treatment of idiopathic pulmonary fibrosis and other interstitial lung diseases in the Nordic countries. Eur Clin Respir J 2015; 2.
Fretheim H, Halse A-K, Seip M, et al. Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort. Rheumatology (Oxford) 2020; 59: 2920–2929.
European Medicines Agency. Orphan designation: overview. 2020. www.ema.europa.eu/en/human-regulatory/ overview/orphan-designation-overview Date last accessed: 18 December 2020.
World Health Organization. WHO report on the global tobacco epidemic 2019: offer help to quit tobacco use [Internet]. 2019. www.who.int/teams/health-promotion/tobacco-control/who-report-on-the-global-tobacco-epidemic-2019 Date last accessed: 2 November 2021.
Navaratnam V, Fleming KM, West J, et al. The rising incidence of idiopathic pulmonary fibrosis in the U.K. Thorax 2011; 66: 462–467.
Jeny F, Brillet P-Y, Kim Y-W, et al. The place of high-resolution computed tomography imaging in the investigation of interstitial lung disease. Expert Rev Respir Med 2019; 13: 79–94.
Hoffmann-Vold A, Maher T, Philpot E, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheumatol 2020; 2: E71–E83.
Organisation for Economic Co-operation and Development. Peer-review of the Finnish shipbuilding industry [Internet]. 2018. www.oecd.org/finland/peer-review-finland-shipbuilding-industry.pdf Date last accessed: 17 December 2020.
Beckett WS. Shipyard workers and asbestos: a persistent and international problem. Occup Environ Med 2007; 64: 639–641.
Associaçao Portuguesa da Cortiça. Information bureau 2019. Cortiça em números. 2018. www.apcor.pt/wp-content/uploads/2019/02/CORTI%c3%87A-em-n%c3%bameros_PT.pdf Date last accessed: 18 December 2020.
Federação Portuguesa de Columbofilia. Sobre a Federação. 2020. www.fpcolumbofilia.pt/index.htm Date last accessed: 17 December 2020.
Nogueira R, Melo N, Novais e Bastos H, et al. Hypersensitivity pneumonitis: antigen diversity and disease implications. Pulmonology 2019; 25: 97–108.
Marques C, Machado D, Marques I, et al. Is probable usual interstitial pneumonia pattern synonymous with idiopathic pulmonary fibrosis? Pulmonology 2019; 25: 253–255.
Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of hypersensitivity pneumonitis in adults. An official ATS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2020; 202: e36–e69.
Varone F, Inoue Y, Richeldi L. Twenty-five years of Respirology: advances in idiopathic pulmonary fibrosis. Respirology 2020; 25: 20–22.
Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 2019; 381: 1718–1727.
Nasser M, Larrieu S, Si-Mohamed S, et al. Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS® study). Eur Respir J 2021; 57: 2002718.
Faverio P, Piluso M, De Giacomi F, et al. Progressive fibrosing interstitial lung diseases: prevalence and characterization in two Italian referral centers. Respiration 2020; 99: 838–845.
Hoffmann-Vold A-M, Allanore Y, Alves M, et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis 2021; 80: 219–227.
Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 2002; 165: 1581–1586.
Perelas A, Silver RM, Arrossi AV, et al. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med 2020; 8: 304–320.
Fischer A, Swigris JJ, Groshong SD, et al. Clinically significant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival. Chest 2008; 134: 601–605.
Kaproth-Joslin K, Croake M, Hobbs S. Update in imaging of idiopathic pulmonary fibrosis. Adv Clin Radiol 2020; 2: 81–100.
Tzilas V, Bouros D. Inherent weaknesses of the current ICD coding system regarding idiopathic pulmonary fibrosis. Eur Respir J 2015; 45: 1194–1196.
