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Review
. 2025 Aug;13(8):739-755.
doi: 10.1016/S2213-2600(25)00129-8. Epub 2025 Jul 17.

Global epidemiology and burden of interstitial lung disease

Paolo Spagnolo  1 , Sabina A Guler  2 , Nazia Chaudhuri  3 , Zarir Udwadia  4 , Lucile Sesé  5 , Bhavika Kaul  6 , Juan I Enghelmayer  7 , Claudia Valenzuela  8 , Atul Malhotra  9 , Christopher J Ryerson  10 , Yet H Khor  11 , Tamera J Corte  12 , Vincent Cottin  13
Affiliations
Review

Global epidemiology and burden of interstitial lung disease

Paolo Spagnolo et al. Lancet Respir Med. 2025 Aug.

Abstract

The global incidence and prevalence of interstitial lung disease (ILD) are difficult to determine due to the rarity of the condition, inconsistent case ascertainment and reporting methods, and differences in risk factors, burden of exposures, and access to health-care systems across geographical areas. With these caveats, several studies have reported an increased incidence and prevalence of ILD over time. Idiopathic pulmonary fibrosis is the most common and extensively studied ILD, whereas connective tissue disease-associated ILD and hypersensitivity pneumonitis display the most geographical variability. Except for idiopathic pulmonary fibrosis, connective tissue disease-associated ILD, hypersensitivity pneumonitis, and sarcoidosis, few data are available for other ILDs. Access to health care remains uneven particularly in low-income and middle-income countries, leading to major health disparities. Socioeconomic inequalities also affect morbidity and mortality within and across countries. We performed a non-systematic review of the most recent literature on the epidemiology and burden of ILD, and highlighted areas where substantial gaps in knowledge remain and further studies are needed. The introduction of new tools, including nationwide health-care databases and monitoring of air pollution exposure, is opening new avenues for ILD epidemiology research; however, we are only at the beginning of understanding how the interaction between genetic and environmental factors contributes to the rising burden of these deadly diseases.

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Conflict of interest statement

Declaration of interests PS reports grants from PPM Services, Roche, Boehringer-Ingelheim, and Chiesi; consulting fees from PPM Services and Novartis; honoraria from Boehringer-Ingelheim; support for attending meetings from PPM Services; and participation on a Data Safety Monitoring Board or Advisory Board for AstraZeneca, Trevi, Merck, Galapagos, Novartis, and Structure Therapeutics. LS reports honoraria for lectures from Boehringer-Ingelheim and AstraZeneca; and support for attending meetings from Oxyvie, Novartis, and Sanofi. CV reports consulting fees from Boehringer-Ingelheim, BMS, and Pliant; honoraria for lectures from Boehringer-Ingelheim and Ferrer; received support for attending meetings for Boehringer-Ingelheim and Ferrer; and participation on a Data Safety Monitoring Board or Advisory Board for Boehringer-Ingelheim and Pliant. AM reports income from Livanova, Eli Lilly, Zoll, and Powell Mansfield unrelated to this manuscript or topic; his laboratory is funded by the US National Institutes of Health and he reports a philantropic donation to the University of California San Diego from ResMed. CJR reports grants from Boehringer-Ingelheim; consulting fees from Boehringer-Ingelheim, Pliant, AstraZeneca, Trevi, Avalyn, and Veracyte; honoraria for lectures from Boehringer-Ingelheim; payment for expert testimony from Boehringer-Ingelheim; and support for attending meetings from Boehringer-Ingelheim and Cipla. YHK reports grants from the National Health and Medical Research Council and Medical Research Future Fund, Austin Medical Research Foundation, Lung Foundation Australia/Thoracic Society of Australia and New Zealand, and Royal Australasian College of Physicians, and in-kind trial support from Air Liquide Healthcare; and consulting fees from Chiesi. YHK is Board Director, Chair for the Clinical Care and Resources Sub-Committee, and OLIV Special Interest Group Convenor for the Thoracic Society of Australia and New Zealand; member of the Clinical Problems Assembly Program Committee and guideline methodologist for the American Thoracic Society; and Associate Editor of the European Respiratory Journal. TJC reports grants from Boehringer-Ingelheim, Bristol Myers Squibb, Roche, Pharmaxis, 4D, Pliant, Bridge Biotherapeutics, and Avalyn Therapeutics; declares consulting fees from Boehringer-Ingelheim, Bristol Myers Squibb, Roche, Pharmaxis, 4D, Pliant, Bridge Biotherapeutics, Avalyn Therapeutics, DevPro, Endeavoiur BioMedicine, and Cincera; reports honoraria for lectures from Bristol Myers Squibb, Roche, and Boehringer-Ingelheim; received support for attending meetings from Bristol Myers Squibb and Boehringer-Ingelheim; and participation on a Data Safety Monitoring Board or Advisory Board for Boehringer-Ingelheim, Bristol Myers Squibb, Roche, Bridge Therapeutics, Avalyn Therapeutics, DevPro, and Endeavor BioMedicines. VC reports consulting fees from Abbvie, AstraZeneca, Avalyn, Boehringer-Ingelheim, Bristol Myers Squibb/Celgene, Ferrer/United Therapeutics, Gossamer, GSK, Liquidia, Pliant, PureTech, Roche, Rolvant, Sanofi, and Shionogi; honoraria for lectures from Boehringer-Ingelheim, Ferrer/United Therapeutics, Roche, and Sanofi; support for attending meetings from Boehringer-Ingelheim and Sanofi; participation on a Data Safety Monitoring Board or Advisory Board for GSK and Molecure; and participation on an adjudication committee for Fibrogen. All other authors declare no competing interests.

Figures

Figure 1:
Figure 1:. Global epidemiology of interstitial lung disease
Diverse data sources contribute to understanding of the evolving and region-specific causes and risk factors that influence the development, progression, and management of interstitial lung disease (ILD). Variability in ILD characteristics, including local epidemiology, subtype, severity, disease behaviour, and available treatment, exist across different global regions. ILD substantially affects individuals and society, leading to reduced life expectancy, poor quality of life, high health-care costs, and substantial burdens on families and caregivers.
Figure 2:
Figure 2:. Global prevalence of idiopathic pulmonary fibrosis
Prevalence ranges include reported estimates across studies in the respective countries, with case definitions ranging from narrow to broad.
See this image and copyright information in PMC

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