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Fernandez, I.E. ; Heinzelmann, K. ; Verleden, S.* ; Eickelberg, O.

Characteristic patterns in the fibrotic lung. Comparing idiopathic pulmonary fibrosis with chronic lung allograft dysfunction.

Ann. Am. Thorac. Soc. 12, S34-S41 (2015)
DOI PMC
Open Access Green as soon as Postprint is submitted to ZB.
Tissue fibrosis, a major cause of death worldwide, leads to significant organ dysfunction in any organ of the human body. In the lung, fibrosis critically impairs gas exchange, tissue oxygenation, and immune function. Idiopathic pulmonary fibrosis (IPF) is the most detrimental and lethal fibrotic disease of the lung, with an estimated median survival of 50% after 3-5 years. Lung transplantation currently remains the only therapeutic alternative for IPF and other end-stage pulmonary disorders. Posttransplant lung function, however, is compromised by short- and long-term complications, most importantly chronic lung allograft dysfunction (CLAD). CLAD affects up to 50% of all transplanted lungs after 5 years, and is characterized by small airway obstruction with pronounced epithelial injury, aberrant wound healing, and subepithelial and interstitial fibrosis. Intriguingly, the mechanisms leading to the fibrotic processes in the engrafted lung exhibit striking similarities to those in IPF; therefore, antifibrotic therapies may contribute to increased graft function and survival in CLAD. In this review, we focus on these common fibrosis-related mechanisms in IPF and CLAD, comparing and contrasting clinical phenotypes, the mechanisms of fibrogenesis, and biomarkers to monitor, predict, or prognosticate disease status.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords Fibrosis ; Idiopathic Pulmonary Fibrosis ; Oclad ; Rclad
ISSN (print) / ISBN 2329-6933
e-ISSN 2325-6621
Journal Annals of the American Thoracic Society
Quellenangaben Volume: 12, Issue: , Pages: S34-S41 Article Number: , Supplement: ,
Publisher American Thoracic Society
Publishing Place New York, NY
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) Institute of Lung Biology (LHI)
Lung Health and Immunity (LHI)