Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena.
idiopathic pulmonary fibrosis
pirfenidone
progression of disease
safety
survival
Journal
Therapeutic advances in respiratory disease
ISSN: 1753-4666
Titre abrégé: Ther Adv Respir Dis
Pays: England
ID NLM: 101316317
Informations de publication
Date de publication:
Historique:
entrez:
19
2
2020
pubmed:
19
2
2020
medline:
2
6
2021
Statut:
ppublish
Résumé
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2-5 years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone. We enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, survival and functional details were collected retrospectively at start of therapy and after 12, 24, 36 and 48 months of treatment. Lung function tests at least 12 months before starting therapy were available for 40 patients and were entered in the database, as well as side effects. During the observation period (922 ± 529 days), 27 patients died, 5 patients underwent lung transplant and 10 patients interrupted therapy due to adverse events or IPF progression. The median survival was 1606 days. There was a significant reduction in disease progression rate, as measured by trend of forced vital capacity, after 1 year of therapy with respect to before treatment ( Our findings confirm the efficacy of pirfenidone in reducing functional progression of IPF and its excellent safety profile in a real-life setting. This study, designed on a long-term follow up, contributes to the growing evidence on safety, tolerability and efficacy of pirfenidone in IPF.
Sections du résumé
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2-5 years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone.
METHODS
We enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, survival and functional details were collected retrospectively at start of therapy and after 12, 24, 36 and 48 months of treatment. Lung function tests at least 12 months before starting therapy were available for 40 patients and were entered in the database, as well as side effects.
RESULTS
During the observation period (922 ± 529 days), 27 patients died, 5 patients underwent lung transplant and 10 patients interrupted therapy due to adverse events or IPF progression. The median survival was 1606 days. There was a significant reduction in disease progression rate, as measured by trend of forced vital capacity, after 1 year of therapy with respect to before treatment (
CONCLUSIONS
Our findings confirm the efficacy of pirfenidone in reducing functional progression of IPF and its excellent safety profile in a real-life setting. This study, designed on a long-term follow up, contributes to the growing evidence on safety, tolerability and efficacy of pirfenidone in IPF.
Identifiants
pubmed: 32066332
doi: 10.1177/1753466620906326
pmc: PMC7029533
doi:
Substances chimiques
Pyridones
0
pirfenidone
D7NLD2JX7U
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1753466620906326Références
Lancet Respir Med. 2017 Jan;5(1):33-41
pubmed: 27876247
Lung. 2017 Oct;195(5):643-651
pubmed: 28674777
Am J Respir Crit Care Med. 2016 Aug 1;194(3):265-75
pubmed: 27299520
Eur Respir Rev. 2017 Dec 6;26(146):
pubmed: 29212837
Front Med (Lausanne). 2019 Mar 13;6:41
pubmed: 30931306
Chest. 2018 Oct;154(4):978-979
pubmed: 30290930
J Clin Med. 2016 Sep 02;5(9):
pubmed: 27598213
JAMA. 2018 Jun 12;319(22):2299-2307
pubmed: 29800034
Eur Respir Rev. 2012 Dec 1;21(126):355-61
pubmed: 23204124
Eur Respir J. 2017 Jan 3;49(1):
pubmed: 28049168
Lancet. 2011 May 21;377(9779):1760-9
pubmed: 21571362
Eur Respir J. 2015 Sep;46(3):795-806
pubmed: 25976683
Med Sci Monit. 2019 Jun 05;25:4193-4201
pubmed: 31166938
Respiration. 2017;94(5):408-415
pubmed: 28898890
J Manag Care Spec Pharm. 2017 Mar;23(3-b Suppl):S17-S24
pubmed: 28287347
Front Med (Lausanne). 2017 Nov 29;4:213
pubmed: 29238708
Pulm Pharmacol Ther. 2017 Aug;45:1-10
pubmed: 28377145
N Engl J Med. 2014 May 29;370(22):2083-92
pubmed: 24836312
Lancet Respir Med. 2020 Jan;8(1):25-33
pubmed: 31575509
Expert Opin Drug Saf. 2016 Nov;15(11):1483-1489
pubmed: 27532218
BMC Pulm Med. 2018 Nov 23;18(1):177
pubmed: 30470213
Pulm Pharmacol Ther. 2018 Jun;50:38-46
pubmed: 29605286
Intern Med. 2018 Oct 1;57(19):2813-2818
pubmed: 29780123
Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824
pubmed: 21471066
Eur Respir J. 2005 Jul;26(1):153-61
pubmed: 15994402
Adv Ther. 2014 Apr;31(4):375-91
pubmed: 24639005
Respir Med. 2015 Jul;109(7):904-13
pubmed: 25962649
Intern Med. 2016;55(5):443-8
pubmed: 26935361
Respir Res. 2019 Jan 21;20(1):16
pubmed: 30665416
Pulmonology. 2019 May - Jun;25(3):149-153
pubmed: 30236523
ERJ Open Res. 2018 Oct 19;4(4):
pubmed: 30364407