title: Where do we stand with IPF treatment?
abstract: Despite 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.
authors.affiliation: 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
authors.forename: C
authors.initials: C
authors.lastname: Albera
authors.forename: C
authors.initials: C
authors.lastname: Ferrero
authors.forename: E
authors.initials: E
authors.lastname: Rindone
authors.forename: S
authors.initials: S
authors.lastname: Zanotto
authors.forename: E
authors.initials: E
authors.lastname: Rizza
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D002986:Clinical Trials as Topic / Q000706:statistics & numerical data*; D019317:Evidence-Based Medicine; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000453:epidemiology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015995:Prevalence; D019141:Respiratory System Agents / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D007166:Immunosuppressive Agents; D019141:Respiratory System Agents