Progress toward improving animal models for idiopathic pulmonary fibrosis.

Degryse AL, Lawson WE
Am J Med Sci. 2011 341 (6): 444-9

PMID: 21613932 · PMCID: PMC3103078 · DOI:10.1097/MAJ.0b013e31821aa000

Idiopathic pulmonary fibrosis (IPF) remains a disease with an unknown cause and a poor prognosis. Among attempts to define disease pathogenesis, animal models of experimental lung fibrosis have a prominent role. Commonly used models include exposure to bleomycin, silica, fluorescein isothiocyanate; irradiation; or expression of specific genes through a viral vector or transgenic system. These all have been instrumental in the study of lung fibrosis, but all have limitations and fall short of recapitulating a pattern of usual interstitial pneumonia, the pathologic correlate to IPF. A model of repetitive bleomycin lung injury has recently been reported that results in marked lung fibrosis, prominent alveolar epithelial cell hyperplasia, a pattern of temporal heterogeneity and persistence of aberrant remodeling well after stimulus removal, representing a significant addition to the collection of animal lung fibrosis models. Taken together, animal models remain a key component in research strategies to better define IPF pathogenesis.

MeSH Terms (7)

Animals Bleomycin Disease Models, Animal Fluorescein-5-isothiocyanate Idiopathic Pulmonary Fibrosis Radiation Injuries, Experimental Silicon Dioxide

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