Idiopathic Pulmonary Fibrosis
Abstract
Idiopathic pulmonary fibrosis appears to be increasing in incidence. It requires early recognition and intervention with supportive care and pharmacologic agents to forestall its progression. Lung transplantation may be curative.
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IPF is marked by interstitial pneumonia, dyspnea and cough, and histologically characterized by fibroblast proliferation and extracellular matrix remodeling.
“marked by interstitial pneumonia, dyspnea and cough25, histologically characterized by fibroblast proliferation and extracellular matrix remodeling26–29.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [25]
IPF is an age-related, progressive lung disease.
“IPF is an age-related, progressive lung disease diagnosed by excluding other potential causes of interstitial lung disease (for example, environmental exposures, connective tissue disease, hypersensitivity pneumonitis or drug toxicities)24 and marked by interstitial pneumonia, dyspnea and cough25, histologically characterized by fibroblast proliferation and extracellular matrix remodeling26–29.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [25]
Idiopathic pulmonary fibrosis is a progressive, fatal, age-associated lung disease with poor survival.
“Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal, age-associated lung disease with poor survival1.”
From Inhaled LTI-03 for idiopathic pulmonary fibrosis: a randomized dose escalation study (2026) · cites this paper as [1]
IPF is a process that is characterized by epithelial cell injury, fibroblast expansion, augmented ECM remodeling, and lung architecture destruction.
“IPF is a process that is characterized by epithelial cell injury, fibroblast expansion, augmented ECM remodeling, and lung architecture destruction (87, 141).”
From Aging and Lung Disease (2020) · cites this paper as [141]
Cites
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- Authors
- Lederer, D. J., & Martinez, F. J.
- Affiliations
- Columbia University Irving Medical Center (US)
- From the Departments of Medicine and Epidemiology, Columbia University Irving Medical Center (D.J.L.), and the Department of Medicine, Weill Cornell Medical Center (F.J.M.) — both in New York.
- ORCID
- Lederer, David J.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 2018-05-10
- Volume
- 378
- Issue
- 19
- Pages
- 1811-1823
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2799479598
- Cited by
- 2,056
- References
- 98
- Citations by year
- 2026: 248 · 2025: 267 · 2024: 299 · 2023: 308 · 2022: 301 · 2021: 266 · 2020: 197 · 2019: 129 · 2018: 40
- FWCI
- 28.43
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Keywords
- idiopathic pulmonary fibrosis, incidence, early recognition, supportive care, pharmacotherapy, disease progression, lung transplantation
- MeSH terms
- Diagnosis, Differential, Dyspnea, Enzyme Inhibitors, Humans, Indoles, Lung, Oxygen Inhalation Therapy, Prognosis, Pyridones, Risk Factors, Tomography, X-Ray Computed, Transforming Growth Factor beta, Idiopathic Pulmonary Fibrosis
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