Cited by 2,020Interstitial Lung Diseases and Idiopathic Pulmonary FibrosisOccupational and environmental lung diseasesPulmonary Hypertension Research and Treatments

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Idiopathic pulmonary fibrosis

Richeldi, Luca, Collard, Harold R, Jones, Mark G·2017·The Lancet

DOI 10.1016/s0140-6736(17)30866-8

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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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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
ORCID: Richeldi, Luca
Collard, Harold R
Jones, Mark G
Venue: The Lancet
Publisher: Elsevier BV
Published: 2017-05
Volume: 389
Issue: 10082
Pages: 1941-1952
ISSN: 0099-5355 (ISSN-L)
0140-6736
DOI: 10.1016/s0140-6736(17)30866-8
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
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
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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