ArticleAdvances in therapy2021
Prevalence and Incidence of Chronic Fibrosing Interstitial Lung Diseases with a Progressive Phenotype in the United States Estimated in a Large Claims Database Analysis.
Article in Advances in therapy, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers, 1 of them a synthesis that pooled it.
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Who cites it
32 citing papers in PubMed, 1 synthesis or guideline pooled it, 52 citations in OpenAlex.
- Incidence and prevalence of interstitial lung diseases worldwide: a systematic literature review.BMJ open respiratory research · 2023Pooled it
- Association ofInternational journal of molecular sciences · 2026Article
- Incidence and prevalence of interstitial lung diseases in Norway: analysis of the national Norwegian patient registry.BMC pulmonary medicine · 2026Article
- Factors associated with delayed diagnosis of fibrotic interstitial lung disease: a retrospective cohort study.BMC pulmonary medicine · 2026Article
- Neuropsychiatric disorders in pulmonary fibrosis: from brain network alterations to inflammatory mechanisms and therapeutic implications.Journal of neuroinflammation · 2026Review
- [Surgical Management of Lung Cancer in Patients with Interstitial Lung Disease].Zhongguo fei ai za zhi = Chinese journal of lung cancer · 2026Review
- Safety and Tolerability of Nintedanib in Japanese Patients with Progressive Fibrosing Interstitial Lung Diseases: Final Results of 2-Year Post-Marketing Surveillance.Advances in therapy · 2026Article
- Health care utilization and costs associated with acute exacerbation of fibrosing interstitial lung disease in the United States: A retrospective administrative claims database analysis.Journal of managed care & specialty pharmacy · 2026Article
- Electronic health record evidence suggests undercoding, underrecognition, and low antifibrotic use in progressive pulmonary fibrosis.PloS one · 2026Article
- Pulmonary Involvement in Autoimmune-Mediated Disease.Deutsches Arzteblatt international · 2025Review
- The importance of considering progression speed in systemic sclerosis-associated interstitial lung diseases: application of 2022 and 2024 clinical practice guidelines for progressive pulmonary fibrosis, a retrospective cohort study.Respiratory research · 2025Article
- Global Burden of Disease of interstitial lung disease and pulmonary sarcoidosis in adolescents and young adults (1990-2019), and projections for the next 30 years.European journal of medical research · 2025Article
- The burden of interstitial lung disease and pulmonary sarcoidosis lung cancer among adolescents and young adults from 1990 to 2021 and its projections: a comparative study between China and other G20 countries.Frontiers in medicine · 2025Article
- Global, regional, and national burden of interstitial lung disease in middle-aged and older adults from 1990 to 2021 with projections to 2050: a systematic analysis of the 2021 Global Burden of Disease Study.Frontiers in medicine · 2025Article
- Frequency of imaging phenotypes of pulmonary interstitial fibrosis.SA journal of radiology · 2025Article
- Interstitial Lung Disease Epidemiology in the Past Three Decades: A Narrative Review.Journal of clinical medicine · 2024Review
- Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease.Thorax · 2024Review
- Article
- Rationale and design of the prognostic transcriptomic signature in fibrotic hypersensitivity pneumonitis (PREDICT) study.ERJ open research · 2024Article
- Article
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Authors and funding
6 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionMany fibrosing interstitial lung diseases (ILDs) develop a chronic progressive phenotype. While idiopathic pulmonary fibrosis, which is always progressive, is well characterized with established treatment options, the epidemiology of other chronic fibrosing ILDs with a progressive phenotype has not been widely investigated. Treatment options are limited, with a high unmet need. This claims database study estimates the incidence and prevalence of these diseases in the USA.
methodsDiagnosis, procedure and resource utilization codes from insurance claims were used to identify patients with fibrosing ILD and those with a chronic progressive phenotype among 37,565,644 adult patients in the IBM
resultsThe estimated age- and sex-adjusted prevalence per 100,000 persons of fibrosing ILD (95% confidence interval) was 117.82 (116.56, 119.08) and of chronic fibrosing ILDs with a progressive phenotype was 70.30 (69.32, 71.27). The estimated adjusted incidence per 100,000 patient-years of fibrosing ILD was 51.56 (50.88, 52.24) and of chronic fibrosing ILDs with a progressive phenotype was 32.55 (32.01, 33.09). Among incident fibrosing ILD patients, 57.3% experienced progression over a median of 117 days (interquartile range 63-224), with largely comparable rates of progression among different diseases.
conclusionsChronic fibrosing ILDs with a progressive phenotype comprise a relatively new disease construct requiring varied approaches to obtain reliable estimates of prevalence and incidence. This is the first large claims database study using real-world data to provide estimates of the prevalence and incidence of these diseases among a very large segment of the US population and could form the groundwork for future studies.
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