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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorGuillen Del Castillo, Alfredo
dc.contributor.authorLópez Meseguer, Manuel
dc.contributor.authorFonollosa Pla, Vicenç
dc.contributor.authorSaez Gimenez, Berta
dc.contributor.authorColunga‑Argüelles, Dolores
dc.contributor.authorRevilla Lopez, Eva Maria
dc.contributor.authorSimeón Aznar, Carmen Pilar
dc.date.accessioned2022-08-12T09:52:41Z
dc.date.available2022-08-12T09:52:41Z
dc.date.issued2022-03-28
dc.identifier.citationGuillén-Del-Castillo A, Meseguer ML, Fonollosa-Pla V, Sáez Giménez B, Colunga-Argüelles D, Revilla-López E, et al. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension. Sci Rep. 2022 Mar 28;12:5289.
dc.identifier.issn2045-2322
dc.identifier.urihttp://hdl.handle.net/11351/7999
dc.descriptionRespiratory tract diseases; Systemic sclerosis
dc.description.abstractTo 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.
dc.language.isoeng
dc.publisherNature Research
dc.relation.ispartofseriesScientific Reports;12
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceScientia
dc.subjectEsclerosi sistemàtica progressiva - Tractament
dc.subjectHipotensió arterial
dc.subjectPulmons - Malalties
dc.subject.meshLung Diseases, Interstitial
dc.subject.meshHypertension, Pulmonary
dc.subject.meshScleroderma, Systemic
dc.titleImpact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1038/s41598-022-09353-z
dc.subject.decsenfermedades pulmonares intersticiales
dc.subject.decshipertensión pulmonar
dc.subject.decsesclerodermia sistémica
dc.relation.publishversionhttps://doi.org/10.1038/s41598-022-09353-z
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Guillén-Del-Castillo A, Fonollosa-Pla V, Simeón-Aznar CP] Unitat de Malalties Autoimmunes, Servei de Medicina Interna, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Meseguer ML, Revilla-López E] Servei de Pneumologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Sáez Giménez B] Servei de Pneumologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Departament de Fisiologia, Universitat Autònoma de Barcelona, Bellaterra, Spain. [Colunga-Argüelles D] Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain
dc.identifier.pmid35347225
dc.identifier.wos000774204500015
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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