Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.

Guillén-Del-Castillo A; Meseguer ML; Fonollosa-Pla V; Giménez BS; Colunga-Argüelles D; Revilla-López E; Rubio-Rivas M; Ropero MJC; Argibay A; Barberá JA; Salas XP; Meñaca AM; Vuelta ABM; Padrón AL; Comet LS; Morera JAD; González-Echávarri C; Mombiela T; Ortego-Centeno N; González MM; Tolosa-Vilella C; Blanco I; Subías PE; Simeón-Aznar CP; RESCLE Consortium; REHAP Consortium

Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.

To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Span...

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Autores:	Guillén-Del-Castillo A, Meseguer ML, Fonollosa-Pla V, Giménez BS, Colunga-Argüelles D, Revilla-López E, Rubio-Rivas M, Ropero MJC, Argibay A, Barberá JA, Salas XP, Meñaca AM, Vuelta ABM, Padrón AL, Comet LS, Morera JAD, González-Echávarri C, Mombiela T, Ortego-Centeno N, González MM, Tolosa-Vilella C, Blanco I, Subías PE, Simeón-Aznar CP, RESCLE Consortium, REHAP Consortium
Tipo de recurso:	artículo
Estado:	Versión publicada
Fecha de publicación:	2022
País:	España
Institución:	Institut d'Investigació i Innovació Parc Taulí (I3PT)
Repositorio:	r-I3PT. Repositorio Institucional Producción Científica del Institut d'Investigació i Innovació Parc Taulí
OAI Identifier:	oai:i3pt.fundanetsuite.com:p1598
Acceso en línea:	https://i3pt.portalinvestigacion.com/publicaciones/1598 https://www.scopus.com/inward/record.uri?eid=2-s2.0-85127250841&doi=10.1038%2fs41598-022-09353-z&partnerID=40&md5=7c5b8193a5d353ae3030ca032a238110
Access Level:	acceso abierto
Palabra clave:	Familial Primary Pulmonary Hypertension Humans Hypertension, Pulmonary Lung Diseases, Interstitial Pulmonary Arterial Hypertension Scleroderma, Systemic human interstitial lung disease pulmonary hypertension systemic sclerosis

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spelling	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.Guillén-Del-Castillo AMeseguer MLFonollosa-Pla VGiménez BSColunga-Argüelles DRevilla-López ERubio-Rivas MRopero MJCArgibay ABarberá JASalas XPMeñaca AMVuelta ABMPadrón ALComet LSMorera JADGonzález-Echávarri CMombiela TOrtego-Centeno NGonzález MMTolosa-Vilella CBlanco ISubías PESimeón-Aznar CPRESCLE ConsortiumREHAP ConsortiumFamilial Primary Pulmonary HypertensionHumansHypertension, PulmonaryLung Diseases, InterstitialPulmonary Arterial HypertensionScleroderma, Systemichumaninterstitial lung diseasepulmonary hypertensionsystemic sclerosisTo assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 ± 5.2 mm vs 19.9 ± 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment.NATURE PORTFOLIO2022info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://i3pt.portalinvestigacion.com/publicaciones/1598https://www.scopus.com/inward/record.uri?eid=2-s2.0-85127250841&doi=10.1038%2fs41598-022-09353-z&partnerID=40&md5=7c5b8193a5d353ae3030ca032a238110Scientific ReportsISSN: 20452322reponame:r-I3PT. Repositorio Institucional Producción Científica del Institut d'Investigació i Innovació Parc Taulíinstname:Institut d'Investigació i Innovació Parc Taulí (I3PT)Inglésinfo:eu-repo/semantics/openAccessoai:i3pt.fundanetsuite.com:p15982026-06-21T15:30:37Z
dc.title.none.fl_str_mv	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
title	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
spellingShingle	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension. Guillén-Del-Castillo A Familial Primary Pulmonary Hypertension Humans Hypertension, Pulmonary Lung Diseases, Interstitial Pulmonary Arterial Hypertension Scleroderma, Systemic human interstitial lung disease pulmonary hypertension systemic sclerosis
title_short	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
title_full	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
title_fullStr	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
title_full_unstemmed	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
title_sort	Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
dc.creator.none.fl_str_mv	Guillén-Del-Castillo A Meseguer ML Fonollosa-Pla V Giménez BS Colunga-Argüelles D Revilla-López E Rubio-Rivas M Ropero MJC Argibay A Barberá JA Salas XP Meñaca AM Vuelta ABM Padrón AL Comet LS Morera JAD González-Echávarri C Mombiela T Ortego-Centeno N González MM Tolosa-Vilella C Blanco I Subías PE Simeón-Aznar CP RESCLE Consortium REHAP Consortium
author	Guillén-Del-Castillo A
author_facet	Guillén-Del-Castillo A Meseguer ML Fonollosa-Pla V Giménez BS Colunga-Argüelles D Revilla-López E Rubio-Rivas M Ropero MJC Argibay A Barberá JA Salas XP Meñaca AM Vuelta ABM Padrón AL Comet LS Morera JAD González-Echávarri C Mombiela T Ortego-Centeno N González MM Tolosa-Vilella C Blanco I Subías PE Simeón-Aznar CP RESCLE Consortium REHAP Consortium
author_role	author
author2	Meseguer ML Fonollosa-Pla V Giménez BS Colunga-Argüelles D Revilla-López E Rubio-Rivas M Ropero MJC Argibay A Barberá JA Salas XP Meñaca AM Vuelta ABM Padrón AL Comet LS Morera JAD González-Echávarri C Mombiela T Ortego-Centeno N González MM Tolosa-Vilella C Blanco I Subías PE Simeón-Aznar CP RESCLE Consortium REHAP Consortium
author2_role	author author author author author author author author author author author author author author author author author author author author author author author author author
dc.subject.none.fl_str_mv	Familial Primary Pulmonary Hypertension Humans Hypertension, Pulmonary Lung Diseases, Interstitial Pulmonary Arterial Hypertension Scleroderma, Systemic human interstitial lung disease pulmonary hypertension systemic sclerosis
topic	Familial Primary Pulmonary Hypertension Humans Hypertension, Pulmonary Lung Diseases, Interstitial Pulmonary Arterial Hypertension Scleroderma, Systemic human interstitial lung disease pulmonary hypertension systemic sclerosis
description	To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 ± 5.2 mm vs 19.9 ± 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment.
publishDate	2022
dc.date.none.fl_str_mv	2022
dc.type.none.fl_str_mv	info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion
format	article
status_str	publishedVersion
dc.identifier.none.fl_str_mv	https://i3pt.portalinvestigacion.com/publicaciones/1598 https://www.scopus.com/inward/record.uri?eid=2-s2.0-85127250841&doi=10.1038%2fs41598-022-09353-z&partnerID=40&md5=7c5b8193a5d353ae3030ca032a238110
url	https://i3pt.portalinvestigacion.com/publicaciones/1598 https://www.scopus.com/inward/record.uri?eid=2-s2.0-85127250841&doi=10.1038%2fs41598-022-09353-z&partnerID=40&md5=7c5b8193a5d353ae3030ca032a238110
dc.language.none.fl_str_mv	Inglés
language_invalid_str_mv	Inglés
dc.rights.none.fl_str_mv	info:eu-repo/semantics/openAccess
eu_rights_str_mv	openAccess
dc.publisher.none.fl_str_mv	NATURE PORTFOLIO
publisher.none.fl_str_mv	NATURE PORTFOLIO
dc.source.none.fl_str_mv	Scientific Reports ISSN: 20452322 reponame:r-I3PT. Repositorio Institucional Producción Científica del Institut d'Investigació i Innovació Parc Taulí instname:Institut d'Investigació i Innovació Parc Taulí (I3PT)
instname_str	Institut d'Investigació i Innovació Parc Taulí (I3PT)
reponame_str	r-I3PT. Repositorio Institucional Producción Científica del Institut d'Investigació i Innovació Parc Taulí
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Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.

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