title: Characteristic patterns in the fibrotic lung. Comparing idiopathic pulmonary fibrosis with chronic lung allograft dysfunction.
abstract: 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.
authors.affiliation: 1 Comprehensive Pneumology Center, University Hospital of Ludwig Maximilians University Munich and Helmholtz Zentrum München, Member of the German Center for Lung Research, Munich, Germany; and.
authors.forename: Isis E
authors.initials: IE
authors.lastname: Fernandez
authors.forename: Katharina
authors.initials: K
authors.lastname: Heinzelmann
authors.forename: Stijn
authors.initials: S
authors.lastname: Verleden
authors.forename: Oliver
authors.initials: O
authors.lastname: Eickelberg
mesh_terms: D000818:Animals; D015415:Biomarkers; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000628:therapy*; D008168:Lung / Q000503:physiopathology*; D016040:Lung Transplantation / Q000009:adverse effects / Q000379:methods*; D051379:Mice; D023421:Models, Animal; D010641:Phenotype; D011379:Prognosis
chemical_list: D015415:Biomarkers
keywords: fibrosis; idiopathic pulmonary fibrosis; oCLAD; rCLAD