PT - JOURNAL ARTICLE AU - Schaefer, C.J. AU - Ruhrmund, D.W. AU - Pan, L. AU - Seiwert, S.D. AU - Kossen, K. 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.