Changes in management of idiopathic pulmonary fibrosis: impact on disease severity and mortality.
Idiopathic pulmonary fibrosis
antifibrotic therapy
interstitial lung disease
mortality
Journal
European clinical respiratory journal
ISSN: 2001-8525
Titre abrégé: Eur Clin Respir J
Pays: United States
ID NLM: 101662134
Informations de publication
Date de publication:
12 Aug 2020
12 Aug 2020
Historique:
entrez:
18
9
2020
pubmed:
19
9
2020
medline:
19
9
2020
Statut:
epublish
Résumé
Idiopathic pulmonary fibrosis (IPF) is a serious interstitial lung disease (ILD) with a median survival of 3-5 years. The aim of the present study was to evaluate disease severity and survival in patients diagnosed with IPF in the era of antifibrotic therapies compared with an earlier IPF cohort. We identified all patients with fibrotic ILD in the hospital electronic case record system between 2011 and 2016, and reviewed each case in order to identify incident patients with IPF. We used the GAP-index to compare disease severity and mortality to previous findings in patients with IPF diagnosed at our center between 2003 and 2009. 260 patients were diagnosed with IPF between 2011 and 2016. Mean age was 72.6 years, 79% were male, mean forced vital capacity (FVC) was 80%, and mean diffusing capacity for carbon monoxide (DLco) was 44%. Age, FVC and DLco were significant predictors of mortality, but the presence of a typical usual interstitial pneumonia pattern on HRCT was not. Eighty percent of patients in GAP stage I received antifibrotic therapy, 73% in GAP stage II, and 29% in GAP stage III.The median survival was four years in the 2011-2016 cohort compared with three years in the 2003-2009 cohort. The distribution of patients between GAP stages was unchanged in 2011-2016 compared with 2003-2009, (stage I 34% vs. 32%, stage II 49% vs. 48% and stage III 20% vs. 16%). One-year mortality was 13% in 2011-2016 and 26% in 2003-2009. In severe disease (GAP stage III), one-year mortality was 26% and 54%, respectively, (p=0.019). Short-term mortality was significantly lower in the 2011-2016 cohort compared with 2003-2009. This improvement may be linked to changes in treatment strategies towards limited use of corticosteroids. Although early diagnosis of IPF still needs increased focus, the improvement is encouraging.
Sections du résumé
BACKGROUND
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is a serious interstitial lung disease (ILD) with a median survival of 3-5 years. The aim of the present study was to evaluate disease severity and survival in patients diagnosed with IPF in the era of antifibrotic therapies compared with an earlier IPF cohort.
METHODS
METHODS
We identified all patients with fibrotic ILD in the hospital electronic case record system between 2011 and 2016, and reviewed each case in order to identify incident patients with IPF. We used the GAP-index to compare disease severity and mortality to previous findings in patients with IPF diagnosed at our center between 2003 and 2009.
RESULTS
RESULTS
260 patients were diagnosed with IPF between 2011 and 2016. Mean age was 72.6 years, 79% were male, mean forced vital capacity (FVC) was 80%, and mean diffusing capacity for carbon monoxide (DLco) was 44%. Age, FVC and DLco were significant predictors of mortality, but the presence of a typical usual interstitial pneumonia pattern on HRCT was not. Eighty percent of patients in GAP stage I received antifibrotic therapy, 73% in GAP stage II, and 29% in GAP stage III.The median survival was four years in the 2011-2016 cohort compared with three years in the 2003-2009 cohort. The distribution of patients between GAP stages was unchanged in 2011-2016 compared with 2003-2009, (stage I 34% vs. 32%, stage II 49% vs. 48% and stage III 20% vs. 16%). One-year mortality was 13% in 2011-2016 and 26% in 2003-2009. In severe disease (GAP stage III), one-year mortality was 26% and 54%, respectively, (p=0.019).
CONCLUSION
CONCLUSIONS
Short-term mortality was significantly lower in the 2011-2016 cohort compared with 2003-2009. This improvement may be linked to changes in treatment strategies towards limited use of corticosteroids. Although early diagnosis of IPF still needs increased focus, the improvement is encouraging.
Identifiants
pubmed: 32944203
doi: 10.1080/20018525.2020.1807682
pii: 1807682
pmc: PMC7480407
doi:
Types de publication
Journal Article
Langues
eng
Pagination
1807682Informations de copyright
© 2020 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.
Déclaration de conflit d'intérêts
The authors have no disclosures.
Références
PLoS One. 2016 Nov 9;11(11):e0166168
pubmed: 27829068
Eur Respir J. 2017 Feb 23;49(2):
pubmed: 28232409
N Engl J Med. 2014 May 29;370(22):2083-92
pubmed: 24836312
BMC Pulm Med. 2020 Jan 8;20(1):3
pubmed: 31914963
Am J Respir Crit Care Med. 2008 Feb 15;177(4):433-9
pubmed: 17975197
Respir Med. 2019 Jul;153:44-51
pubmed: 31153107
N Engl J Med. 2014 May 29;370(22):2071-82
pubmed: 24836310
Lancet. 2011 May 21;377(9779):1760-9
pubmed: 21571362
BMC Pulm Med. 2018 Jan 17;18(1):9
pubmed: 29343236
N Engl J Med. 2012 May 24;366(21):1968-77
pubmed: 22607134
Am J Respir Crit Care Med. 2017 Jan 1;195(1):78-85
pubmed: 27331880
BMC Pulm Med. 2017 Sep 15;17(1):124
pubmed: 28915874
Eur Respir J. 2015 Jul;46(1):186-96
pubmed: 25837040
Respir Res. 2019 Mar 12;20(1):55
pubmed: 30866942
Multidiscip Respir Med. 2018 May 14;13:14
pubmed: 29785264
Respir Med. 2015 Apr;109(4):510-6
pubmed: 25736347
ERJ Open Res. 2019 Jul 08;5(3):
pubmed: 31304177
Respir Res. 2019 May 24;20(1):103
pubmed: 31126287
Ann Intern Med. 2012 May 15;156(10):684-91
pubmed: 22586007
Respir Med. 2014 Apr;108(4):647-53
pubmed: 24529739
AJR Am J Roentgenol. 2013 May;200(5):W458-67
pubmed: 23617514
Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824
pubmed: 21471066
Respir Res. 2019 Sep 6;20(1):205
pubmed: 31492155
Respir Med. 2014 May;108(5):793-9
pubmed: 24636811
Lancet Respir Med. 2018 Feb;6(2):138-153
pubmed: 29154106
Respir Res. 2019 Jan 21;20(1):16
pubmed: 30665416