Evidence map›Paper›PMID 34233665›Full record

ReviewRespiratory research2021

Global incidence and prevalence of idiopathic pulmonary fibrosis.

Toby M Maher, Elisabeth Bendstrup, Louis Dron, Jonathan Langley, Gerald Smith, Javaria Mona Khalid, Haridarshan Patel, Michael Kreuter

Abstract readReview
In one paragraph

Review in Respiratory research, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 366 papers, 11 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
366citing papers in PubMed, 11 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

366 citing papers in PubMed, 11 syntheses or guidelines pooled it.

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  3. Comorbidities in clinical cohorts of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis.European respiratory review : an official journal of the European Respiratory Society · 2026
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  11. Lack of diversity in antifibrotic trials for pulmonary fibrosis: a systematic review.European respiratory review : an official journal of the European Respiratory Society · 2025
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  14. Bexotegrast Shows Dose-Dependent Integrin αAnnals of the American Thoracic Society · 2025
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  20. Postoperative acute exacerbations in patients with idiopathic pulmonary fibrosis undergoing lung cancer surgery.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2026
    Article

306 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Toby M MaherKeck School of Medicine, The University of Southern California, Los Angeles, CA, USA. toby.maher@med.usc.edu.ORCID http://orcid.org/0000-0001-7192-9149
Elisabeth BendstrupCenter for Rare Lung Diseases, Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark.
Louis DronCytel, Vancouver, BC, Canada.
Jonathan LangleyGlobal Medical Affairs, Galapagos, NV, Mechelen, Belgium.
Gerald SmithCytel, Vancouver, BC, Canada.
Javaria Mona KhalidEvidence Generation and Epidemiology, Global Medical Affairs, Galapagos NV, Mechelen, Belgium.
Haridarshan PatelEvidence Generation and Epidemiology, Global Medical Affairs, Galapagos NV, Mechelen, Belgium.
Michael KreuterCenter for Interstitial and Rare Lung Diseases, Department of Pneumology and Respiratory Critical Care Medicine, University of Heidelberg, Heidelberg, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

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.

Indexed as

Global HealthHumansIdiopathic Pulmonary FibrosisIncidenceObservational Studies as TopicPopulation SurveillancePrevalenceCase definitionEpidemiologyIdiopathic pulmonary fibrosisInterstitial lung diseaseModelingRare disease

Identifiers

PMID34233665
PMCPMC8261998

What OpenQuestion holds

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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.