The DOI matches its Crossref record; 141 of the 143 references it cites were found in a registry or an index.
Idiopathic pulmonary fibrosis
Abstract
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death. In less than a decade, understanding of the pathogenesis and management of this disease has been transformed, and two disease-modifying therapies have been approved, worldwide. In this Seminar, we summarise the presentation, pathophysiology, diagnosis, and treatment options available for patients with idiopathic pulmonary fibrosis. This disease has improved understanding of the mechanisms of lung fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
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“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 [26]
In the citing paper
“The median survival time after diagnosis is 2–4 years despite widespread availability of targeted antifibrotic therapies26,30, highlighting the ongoing need for novel, effective therapies to treat IPF.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [26]
In the citing paper
“Clinical trials investigating these two drugs in patients with IPF have demonstrated only a slowing of disease progression33,34, as measured by forced vital capacity (FVC) and self-reported quality of life (QOL) metrics, and unclear benefit to survival26, making the development of therapies that can restore lung function and reverse the course of disease a priority.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [26]
In the citing paper
“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 [26]
In the citing paper
“These observations indicate that the Pdgf pathway is crucial to disease pathogenesis.”
From Reference-based analysis of lung single-cell sequencing reveals a transitional profibrotic macrophage (2019) · cites this paper as [39]
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Metadata
- Authors
- Richeldi, L., Collard, H. R., & Jones, M. G.
- Affiliations
- Università Cattolica del Sacro Cuore (IT)
- University of California, San Francisco (US)
- National Institute for Health and Care Research (GB)
- Corresponding author
- Richeldi, Luca
- Venue
- The Lancet
- Publisher
- Elsevier BV
- Published
- 2017-05
- Volume
- 389
- Issue
- 10082
- Pages
- 1941-1952
- ISSN
- 0099-5355 (ISSN-L)
- 0140-6736
- OpenAlex
- W2599075603
- Cited by
- 2,020
- References
- 143
- Citations by year
- 2026: 202 · 2025: 317 · 2024: 338 · 2023: 293 · 2022: 273 · 2021: 233 · 2020: 167 · 2019: 94 · 2018: 76 · 2017: 22
- FWCI
- 17.97
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Lancet (publishedVersion)
- PubMed (publishedVersion)
- ePrints Soton (University of Southampton) (acceptedVersion)
- Funders
- National Institute for Health and Care Research (CL-2016-26-004)
- 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 › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- idiopathic pulmonary fibrosis, pulmonary fibrosis, pathophysiology, extracellular matrix, alveolar architecture, disease-modifying therapy, diagnosis, treatment options, respiratory failure
- MeSH terms
- Humans, Idiopathic Pulmonary Fibrosis
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