Authorship and contributorship
Pirfenidone: significant treatment effects in idiopathic pulmonary fibrosis
Version of Record online: 14 JUN 2012
© 2012 Blackwell Publishing Ltd
The Clinical Respiratory Journal
Volume 6, Issue 3, pages 131–143, July 2012
How to Cite
Hilberg, O., Simonsen, U., du Bois, R. and Bendstrup, E. (2012), Pirfenidone: significant treatment effects in idiopathic pulmonary fibrosis. The Clinical Respiratory Journal, 6: 131–143. doi: 10.1111/j.1752-699X.2012.00302.x
All authors wrote and revised the paper
Conflict of interest
The authors have stated explicitly that there are no conflicts of interest in connection with this article.
- Issue online: 14 JUN 2012
- Version of Record online: 14 JUN 2012
- Manuscript Accepted: 31 MAY 2012
- Manuscript Received: 30 MAY 2012
- Manuscript Revised: 30 MAY 2012
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 Europe and elsewhere for the treatment of IPF. Along with the new ATS/ERS/JRS/ALAT 2011 statement for ‘Evidence Based Guidelines for Diagnosis and Management’, 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.