PT  - JOURNAL ARTICLE
AU  - C.J. Schaefer
AU  - D.W. Ruhrmund
AU  - L. Pan
AU  - S.D. Seiwert
AU  - K. Kossen
TI  - Antifibrotic activities of pirfenidone in animal models
AID  - 10.1183/09059180.00001111
DP  - 2011 Jun 01
TA  - European Respiratory Review
PG  - 85--97
VI  - 20
IP  - 120
4099  - http://err.ersjournals.com/content/20/120/85.short
4100  - http://err.ersjournals.com/content/20/120/85.full
SO  - EUROPEAN RESPIRATORY REVIEW2011 Jun 01; 20
AB  - Pirfenidone is an orally active small molecule that has recently been evaluated in large clinical trials for the treatment of idiopathic pulmonary fibrosis, a fatal disease in which the uncontrolled deposition of extracellular matrix leads to progressive loss of lung function. This review describes the activity of pirfenidone in several well-characterised animal models of fibrosis in the lung, liver, heart and kidney. In these studies, treatment-related reductions in fibrosis are associated with modulation of cytokines and growth factors, with the most commonly reported effect being reduction of transforming growth factor-β. The consistent antifibrotic activity of pirfenidone in a broad array of animal models provides a strong preclinical rationale for the clinical characterisation of pirfenidone in pulmonary fibrosis and, potentially, other conditions with a significant fibrotic component.