RT Journal Article
SR Electronic
T1 Antifibrotic activities of pirfenidone in animal models
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 85
OP 97
DO 10.1183/09059180.00001111
VO 20
IS 120
A1 Schaefer, C.J.
A1 Ruhrmund, D.W.
A1 Pan, L.
A1 Seiwert, S.D.
A1 Kossen, K.
YR 2011
UL http://err.ersjournals.com/content/20/120/85.abstract
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.