Global incidence and prevalence of idiopathic pulmonary fibrosis

Respiratory Research

Michael Kreuter¹  Elisabeth Bendstrup²  Louis Dron³  Gerald Smith³  Javaria Mona Khalid⁴  Haridarshan Patel⁴  Jonathan Langley⁵  Toby M. Maher⁶ 

[1] Center for Interstitial and Rare Lung Diseases, Department of Pneumology and Respiratory Critical Care Medicine, University of Heidelberg, Heidelberg, Germany;German Center for Lung Research, Heidelberg, Germany;Center for Rare Lung Diseases, Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark;Cytel, Vancouver, BC, Canada;Evidence Generation and Epidemiology, Global Medical Affairs, Galapagos NV, Mechelen, Belgium;Global Medical Affairs, Galapagos, NV, Mechelen, Belgium;Keck School of Medicine, The University of Southern California, Los Angeles, CA, USA;National Heart and Lung Institute, Imperial College, London, UK;

关键词: Case definition;    Epidemiology;    Idiopathic pulmonary fibrosis;    Interstitial lung disease;    Modeling;    Rare disease;   

DOI  :  10.1186/s12931-021-01791-z

来源: Springer

【 摘 要 】

BackgroundIdiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease with considerable morbidity. Heterogeneity in epidemiologic studies means the full impact of the disease is unclear.MethodsA targeted literature search for population-based, observational studies reporting incidence and/or prevalence of IPF from January 2009 to April 2020 was conducted. Identified studies were aggregated by country. For countries with multiple publications, a weighted average was determined. Incidence and prevalence data were adjusted for between-study differences where possible. The final model included adjusted estimates of incidence and prevalence per 10,000 of the population with 95% confidence intervals. As prevalence estimates vary depending on the definitions used, estimates were based on a specific case definition of IPF.ResultsOverall, 22 studies covering 12 countries met the inclusion criteria, with 15 reporting incidence and 18 reporting prevalence estimates. The adjusted incidence estimates (per 10,000 of the population) ranged from 0.35 to 1.30 in Asia–Pacific countries, 0.09 to 0.49 in Europe, and 0.75 to 0.93 in North America. Unadjusted and adjusted incidence estimates were consistent. The adjusted prevalence estimates ranged from 0.57 to 4.51 in Asia–Pacific countries, 0.33 to 2.51 in Europe, and 2.40 to 2.98 in North America. South Korea had the highest incidence and prevalence estimates. When prevalence estimates were compared to country-specific rare disease thresholds, IPF met the definition of a rare disease in all countries except South Korea. There were notable geographic gaps for IPF epidemiologic data.ConclusionsDue to differences in study methodologies, there is worldwide variability in the reported incidence and prevalence of IPF. Based on the countries included in our analysis, we estimated the adjusted incidence and prevalence of IPF to be in the range of 0.09–1.30 and 0.33–4.51 per 10,000 persons, respectively. According to these prevalence estimates, IPF remains a rare disease. For consistency, future epidemiologic studies of IPF should take age, sex, smoking status, and the specificity of case definitions into consideration.

【 授权许可】

CC BY