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Updated: Aug 7, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
07:51

Refined Murine Model of Idiopathic Pulmonary Fibrosis

Published on: June 17, 2025

[Idiopathic pulmonary fibrosis (IPF)]

Aki Matsumoto1, Arata Azuma

  • 1Fourth Department of Internal Medicine, Nippon Medical School.

Nihon Rinsho. Japanese Journal of Clinical Medicine
|July 15, 2006
PubMed

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disease that prognosis (INF) has not improved by available therapy. Recently, much attention has focused on the utility of the T-helper type 1 cytokine interferon (IFN)-gamma as a treatment for IPF. The rationale for its use has been based on observations of its properties as an inhibitor of fibroblast proliferation, collagen synthesis and deposition, and expression of profibrotic cytokines. The clinical trial is advanced aiming at the prognosis improvement of IPF patients. Small-scale controlled clinical study in 1999, IFN-gamma has improved the respiratory functions of IPF, which was resistant to steroid therapy. Be based on these results, phase III large-scale study mainly in North America, the lagged effect of respiratory function aggravation was expected in IPF, in which pulmonary functions are mildly or moderately deteriolated. But in progressive cases, interferon gamma-1b did not affect progression-free survival, pulmonary function, or the quality of life.

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