Exploring animal models that resemble idiopathic pulmonary fibrosis

Jun Tashiro, Gustavo A. Rubio, Andrew H. Limper, Kurt Williams, Sharon J. Elliot, Ioanna Ninou, Vassilis Aidinis, Argyrios Tzouvelekis, Marilyn K. Glassberg

Research output: Contribution to journalReview articlepeer-review

211 Scopus citations

Abstract

Large multicenter clinical trials have led to two recently approved drugs for patients with idiopathic pulmonary fibrosis (IPF); yet, both of these therapies only slow disease progression and do not provide a definitive cure. Traditionally, preclinical trials have utilized mouse models of bleomycin (BLM)-induced pulmonary fibrosis-though several limitations prevent direct translation to human IPF. Spontaneous pulmonary fibrosis occurs in other animal species, including dogs, horses, donkeys, and cats. While the fibrotic lungs of these animals share many characteristics with lungs of patients with IPF, current veterinary classifications of fibrotic lung disease are not entirely equivalent. Additional studies that profile these examples of spontaneous fibroses in animals for similarities to human IPF should prove useful for both human and animal investigators. In the meantime, studies of BLM-induced fibrosis in aged male mice remain the most clinically relevant model for preclinical study for human IPF. Addressing issues such as time course of treatment, animal size and characteristics, clinically irrelevant treatment endpoints, and reproducibility of therapeutic outcomes will improve the current status of preclinical studies. Elucidating the mechanisms responsible for the development of fibrosis and disrepair associated with aging through a collaborative approach between researchers will promote the development of models that more accurately represent the realm of interstitial lung diseases in humans.

Original languageEnglish (US)
Article number118
JournalFrontiers in Medicine
Volume4
Issue numberJUL
DOIs
StatePublished - 2017
Externally publishedYes

Keywords

  • Aged mice
  • Asbestosis
  • Bleomycin
  • Idiopathic pulmonary fibrosis
  • Murine model

ASJC Scopus subject areas

  • General Medicine

Fingerprint

Dive into the research topics of 'Exploring animal models that resemble idiopathic pulmonary fibrosis'. Together they form a unique fingerprint.

Cite this