• C Albera, C Ferrero, E Rindone, S Zanotto, and E Rizza.
• Università di Torino, Facoltà di Medicina e Chirurgia San Luigi Gonzaga, Dipartimento di Scienze Cliniche e Biologiche Scuola di Specializzazione in Malattie dell'Apparato Respiratorio, Torino, Italy. carlo.albera@unito.it
• Resp Res. 2013 Jan 1;14 Suppl 1:S7.

AbstractDespite receiving 'weak no' recommendations in the updated guidelines on treating patients with Idiopathic Pulmonary Fibrosis (IPF), two key treatment options are pirfenidone and N-acetylcysteine (NAC), and both are used in clinical practice. The efficacy of pirfenidone is supported by a number of Phase III trials as well as a Cochrane meta-analysis. Tolerability data are also provided by clinical trials and a long-term extension phase of these studies. Pirfenidone is approved in Europe for the treatment of patients with mild-to-moderate IPF. NAC-based therapy has no such approval, but is commonly used to treat patients. A Phase III trial suggested some benefit of the NAC, prednisone and azathioprine regimen for IPF patients, but the study had many limitations. A further study to investigate this regimen, compared with a placebo alone arm, was recently stopped due to increased mortality in the triple-therapy arm. Discussion of these data and recent findings highlight the importance of a further update to the existing guidelines, so that IPF specialists can provide the most up-to-date advice and treatment to patients in clinical practice.

18 keywords...

hide…