Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry.
Anti-fibrotic treatment
Idiopathic pulmonary fibrosis
National registry
SEPAR
Spain
Journal
Respiratory research
ISSN: 1465-993X
Titre abrégé: Respir Res
Pays: England
ID NLM: 101090633
Informations de publication
Date de publication:
17 Jun 2019
17 Jun 2019
Historique:
received:
07
02
2019
accepted:
24
05
2019
entrez:
19
6
2019
pubmed:
19
6
2019
medline:
7
1
2020
Statut:
epublish
Résumé
Little is known on the characteristics of patients diagnosed with idiopathic pulmonary fibrosis (IPF) in Spain. We aimed to characterize the demographic and clinical profile of IPF patients included in the IPF National Registry of the Spanish Respiratory Society (SEPAR). This is a prospective, observational, multicentre and nationwide study that involved 608 IPF patients included in the SEPAR IPF Registry up to June 27th, 2017, and who received any treatment for their disease. IPF patients were predominantly males, ex-smokers, and aged in their 70s, similar to other registries. Upon inclusion, mean ± SD predicted forced vital capacity was 77.6% ± 19.4, diffusing capacity for carbon monoxide was 48.5% ± 17.7, and the 6-min walk distance was 423.5 m ± 110.4. The diagnosis was mainly established on results from the high-resolution computed tomography in the proper clinical context (55.0% of patients), while 21.2% of patients required invasive procedures (surgical lung biopsy) for definitive diagnosis. Anti-fibrotic treatment was prescribed in 69.4% of cases, 51.5% pirfenidone and 17.9% nintedanib, overall with a good safety profile. The SEPAR IPF Registry should help to further characterize current characteristics and future trends of IPF patients in Spain and compare/pool them with other registries and cohorts.
Sections du résumé
BACKGROUND
BACKGROUND
Little is known on the characteristics of patients diagnosed with idiopathic pulmonary fibrosis (IPF) in Spain. We aimed to characterize the demographic and clinical profile of IPF patients included in the IPF National Registry of the Spanish Respiratory Society (SEPAR).
METHODS
METHODS
This is a prospective, observational, multicentre and nationwide study that involved 608 IPF patients included in the SEPAR IPF Registry up to June 27th, 2017, and who received any treatment for their disease. IPF patients were predominantly males, ex-smokers, and aged in their 70s, similar to other registries.
RESULTS
RESULTS
Upon inclusion, mean ± SD predicted forced vital capacity was 77.6% ± 19.4, diffusing capacity for carbon monoxide was 48.5% ± 17.7, and the 6-min walk distance was 423.5 m ± 110.4. The diagnosis was mainly established on results from the high-resolution computed tomography in the proper clinical context (55.0% of patients), while 21.2% of patients required invasive procedures (surgical lung biopsy) for definitive diagnosis. Anti-fibrotic treatment was prescribed in 69.4% of cases, 51.5% pirfenidone and 17.9% nintedanib, overall with a good safety profile.
CONCLUSIONS
CONCLUSIONS
The SEPAR IPF Registry should help to further characterize current characteristics and future trends of IPF patients in Spain and compare/pool them with other registries and cohorts.
Identifiants
pubmed: 31208406
doi: 10.1186/s12931-019-1084-0
pii: 10.1186/s12931-019-1084-0
pmc: PMC6580511
doi:
Substances chimiques
Anti-Inflammatory Agents, Non-Steroidal
0
Pyridones
0
pirfenidone
D7NLD2JX7U
Types de publication
Journal Article
Multicenter Study
Observational Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
127Investigateurs
M Aburto Barrenetxea
(M)
I Alfageme Michavila
(I)
E Bollo de Miguel
(E)
E Cano
(E)
A Casanova Espinosa
(A)
D Castillo Villegas
(D)
J A Figuerola Mendal
(JA)
R García Sevila
(R)
J I Gaudó Navarro
(JI)
L Gómez Carrera
(L)
J M González
(JM)
S Herrera Lara
(S)
R Laporta Hernández
(R)
M Marín González
(M)
M A Nieto Barbero
(MA)
K Portillo
(K)
A D Romero
(AD)
R Sánchez Simón-Talero
(R)
J N Sancho Chust
(JN)
J Sellarés Torres
(J)
M J Soler Sempere
(MJ)
J Sauleda Roig
(J)
L Tomás López
(L)
M Villanueva Montes
(M)
Références
Am J Respir Crit Care Med. 2001 Oct 1;164(7):1171-81
pubmed: 11673205
Am J Respir Crit Care Med. 2003 Apr 1;167(7):962-9
pubmed: 12663338
Ann Intern Med. 2005 Jun 21;142(12 Pt 1):963-7
pubmed: 15968010
Eur Respir J. 2006 Jan;27(1):136-42
pubmed: 16387946
Chest. 2007 May;131(5):1448-53
pubmed: 17400656
Am J Respir Crit Care Med. 2008 Feb 15;177(4):433-9
pubmed: 17975197
Respir Med. 2010 Jul;104(7):1035-41
pubmed: 20199856
Am J Respir Crit Care Med. 2011 Feb 15;183(4):431-40
pubmed: 20935110
Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824
pubmed: 21471066
Lancet. 2011 May 21;377(9779):1760-9
pubmed: 21571362
Thorax. 2012 Mar;67(3):264; author reply 264-5
pubmed: 21900698
Am J Respir Crit Care Med. 2011 Dec 15;184(12):1382-9
pubmed: 21940789
Ann Intern Med. 2012 May 15;156(10):684-91
pubmed: 22586007
Eur Respir J. 2013 May;41(5):1207-18
pubmed: 23100500
Eur Respir J. 2013 Oct;42(4):916-23
pubmed: 23598958
Arch Bronconeumol. 2013 Aug;49(8):343-53
pubmed: 23742884
Eur Respir Rev. 2013 Sep 1;22(129):281-91
pubmed: 23997056
Clin Epidemiol. 2013 Nov 25;5:483-92
pubmed: 24348069
Lancet Respir Med. 2013 Jul;1(5):369-76
pubmed: 24429201
Lancet Respir Med. 2014 Apr;2(4):277-84
pubmed: 24717624
N Engl J Med. 2014 May 29;370(22):2083-92
pubmed: 24836312
Eur Respir J. 2014 Aug;44(2):273-6
pubmed: 25082902
Chest. 2015 Feb;147(2):450-459
pubmed: 25317858
Respir Med. 2015 Jun;109(6):661-70
pubmed: 25727856
Eur Respir J. 2015 Jul;46(1):186-96
pubmed: 25837040
Respiration. 2015;90(4):352
pubmed: 26278125
BMC Pulm Med. 2015 Aug 19;15:92
pubmed: 26285574
Eur Respir J. 2015 Oct;46(4):1113-30
pubmed: 26424523
Thorax. 2016 May;71(5):429-35
pubmed: 26968970
Lancet Respir Med. 2016 May;4(5):381-9
pubmed: 27050871
Eur Clin Respir J. 2016 Apr 21;3:31090
pubmed: 27105945
Am J Respir Crit Care Med. 2017 Mar 15;195(6):801-813
pubmed: 27684041
Eur Respir J. 2017 Feb 23;49(2):
pubmed: 28232409
Lancet. 2017 May 13;389(10082):1941-1952
pubmed: 28365056
Eur Respir J. 2017 Aug 31;50(2):
pubmed: 28860269
Clin Respir J. 2018 Apr;12(4):1526-1535
pubmed: 28862397
BMC Pulm Med. 2017 Sep 15;17(1):124
pubmed: 28915874
Eur Respir Rev. 2017 Oct 3;26(145):null
pubmed: 28974541
Lancet Respir Med. 2018 Feb;6(2):138-153
pubmed: 29154106
Respir Res. 2018 Feb 22;19(1):32
pubmed: 29471816
BMC Pulm Med. 2018 May 21;18(1):75
pubmed: 29784045
Respir Res. 2018 Jul 28;19(1):141
pubmed: 30055613
Respiration. 2018;96(6):514-524
pubmed: 30114692
Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-e68
pubmed: 30168753
Chest. 1997 Jan;111(1):51-7
pubmed: 8995992