Open AccessPirfenidone: significant treatment effects in idiopathic pulmonary fibrosis
Author(s) -
Hilberg Ole,
Simonsen Ulf,
du Bois Roland,
Bendstrup Elisabeth
Publication year - 2012
Publication title -
the clinical respiratory journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.789
H-Index - 33
eISSN - 1752-699X
pISSN - 1752-6981
DOI - 10.1111/j.1752-699x.2012.00302.x
Subject(s) - pirfenidone , medicine , idiopathic pulmonary fibrosis , pulmonary fibrosis , lung , fibrosis , interstitial lung disease , intensive care medicine , clinical trial
Pirfenidone has been shown in three recently published trials to slow down the progression of the devastating interstitial lung disease, idiopathic pulmonary fibrosis ( IPF ). The precise mechanisms that initiate and perpetuate the histopathological process leading to lung fibrosis in IPF are still uncertain, but increased concentrations of reactive oxidative species and fibrogenetic factors have been observed in the pulmonary tissue of patients. Although the exact mechanisms of its action are unknown, pirfenidone is a small molecule with antifibrotic and some hydroxyl scavenger properties that has recently been approved in E urope and elsewhere for the treatment of IPF . Along with the new ATS / ERS / JRS / ALAT 2011 statement for ‘ E vidence B ased G uidelines for D iagnosis and M anagement’, there is now a more profound basis for offering IPF patients an evidence‐based evaluation and treatment. This review summarizes the background to the recommended use of pirfenidone for the treatment of IPF . Please cite this paper as: Hilberg O, Simonsen U, du Bois R and Bendstrup E. Pirfenidone: significant treatment effects in idiopathic pulmonary fibrosis. Clin Respir J 2012; 6: 131–143.