TY - JOUR T1 - Antifibrotic activities of pirfenidone in animal models JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 85 LP - 97 DO - 10.1183/09059180.00001111 VL - 20 IS - 120 AU - C.J. Schaefer AU - D.W. Ruhrmund AU - L. Pan AU - S.D. Seiwert AU - K. Kossen Y1 - 2011/06/01 UR - http://err.ersjournals.com/content/20/120/85.abstract N2 - 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. ER -