Cited by 595Interstitial Lung Diseases and Idiopathic Pulmonary FibrosisSystemic Sclerosis and Related DiseasesPulmonary Hypertension Research and Treatments

Pulmonary fibrosis: patterns and perpetrators

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Noble, Paul W., Barkauskas, Christina E., Jiang, Dianhua·2012·Journal of Clinical Investigation

Abstract

Pulmonary fibrosis occurs in a variety of clinical settings, constitutes a major cause of morbidity and mortality, and represents an enormous unmet medical need. However, the disease is heterogeneous, and the failure to accurately discern between forms of fibrosing lung diseases leads to inaccurate treatments. Pulmonary fibrosis occurring in the context of connective tissue diseases is often characterized by a distinct pattern of tissue pathology and may be amenable to immunosuppressive therapies. In contrast, idiopathic pulmonary fibrosis (IPF) is a progressive and lethal form of fibrosing lung disease that is recalcitrant to therapies that target the immune system. Although animal models of fibrosis imperfectly recapitulate IPF, they have yielded numerous targets for therapeutic intervention. Understanding the heterogeneity of these diseases and elucidating the final common pathways of fibrogenesis are critical for the development of efficacious therapies for severe fibrosing lung diseases.

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Research works printed but not linked: 2

  1. King TE Jr, Pardo A, Selman M. Idiopathic pulmo- nary fibrosis. Lancet. 2011;378(9807):1949-1961.
  2. White ES, Lazar MH, Thannickal VJ. Pathoge- netic mechanisms in usual interstitial pneumo- nia/idiopathic pulmonary fibrosis. J Pathol. 2003; 201(3):343-354.

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Authors
Noble, P. W., Barkauskas, C. E., & Jiang, D.
Affiliations
Duke University (US)
Corresponding author
Noble, Paul W.
Venue
Journal of Clinical Investigation
Published
2012-08-01
Volume
122
Issue
8
Pages
2756-2762
ISSN
0021-9738 (ISSN-L)
OpenAlex
W2116163278
Cited by
595
References
106
Citations by year
2026: 38 · 2025: 55 · 2024: 51 · 2023: 70 · 2022: 51 · 2021: 43 · 2020: 39 · 2019: 36 · 2018: 31 · 2017: 41 · 2016: 33 · 2015: 33 · 2014: 45 · 2013: 24 · 2012: 3
FWCI
5.59
Type
journal article
Language
en
Open access
Locations
  • Journal of Clinical Investigation (publishedVersion)
  • Europe PMC (PubMed Central) (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • National Institutes of Health (5p50hl084917-05)
  • School of Medicine, Duke University
  • National Heart, Lung, and Blood Institute (P01HL108793, R01HL077291, P50-HL084917, R01HL060539)
  • National Institute of Allergy and Infectious Diseases (AI052201, R01AI052201)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
Keywords
pulmonary fibrosis, idiopathic pulmonary fibrosis, fibrosing lung disease, disease heterogeneity, immunosuppressive therapy, fibrogenesis, therapeutic target
MeSH terms
Animals, Humans, Mesoderm, Models, Biological, Pulmonary Alveoli, Pulmonary Fibrosis, Genetic Predisposition to Disease, Respiratory Mucosa, Idiopathic Pulmonary Fibrosis

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