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DC Field | Value | Language |
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dc.contributor.author | Guillén del Castillo, Alfredo | - |
dc.contributor.author | López Meseguer, Manuel | - |
dc.contributor.author | Fonollosa Pla, Vicent | - |
dc.contributor.author | Sáez Giménez, Berta | - |
dc.contributor.author | Colunga Argüelles, Dolores | - |
dc.contributor.author | Revilla López, Eva | - |
dc.contributor.author | Rubio Rivas, Manuel | - |
dc.contributor.author | Cristo Ropero, Maria Jose | - |
dc.contributor.author | Argibay, Ana | - |
dc.contributor.author | Barberà i Mir, Joan Albert | - |
dc.contributor.author | Pla Salas, Xavier | - |
dc.contributor.author | Martínez Meñaca, Amaya | - |
dc.contributor.author | Madroñero Vuelta, Ana Belén | - |
dc.contributor.author | Lara Padrón, Antonio | - |
dc.contributor.author | Sáez Comet, Luis | - |
dc.contributor.author | Domingo Morera, Juan Antonio | - |
dc.contributor.author | González Echávarri, Cristina | - |
dc.contributor.author | Mombiela, Teresa | - |
dc.contributor.author | Ortego Centeno, Norberto | - |
dc.contributor.author | Marín González, Manuela | - |
dc.contributor.author | Tolosa Vilella, Carles | - |
dc.contributor.author | Blanco Vich, Isabel | - |
dc.contributor.author | Escribano Subias, Pilar | - |
dc.contributor.author | Simeón Aznar, Carmen Pilar | - |
dc.contributor.author | Aurtenetxe Pérez, Águeda | - |
dc.contributor.author | Barberà i Mir, Joan Albert | - |
dc.contributor.author | Barrios Garrido-Lestache, Elvira | - |
dc.contributor.author | Bedate Díaz, Pedro | - |
dc.contributor.author | Cifrián, José Manuel | - |
dc.contributor.author | Dos Subirá, Laura | - |
dc.contributor.author | Hernández, Teresa Elías | - |
dc.contributor.author | García Hernández, Francisco José | - |
dc.contributor.author | Gil Carbonell, Juan | - |
dc.contributor.author | González Segovia, Ariadna | - |
dc.contributor.author | Hermida Valverde, Tamara | - |
dc.contributor.author | Hernández Baldomero, Idaira Fámara | - |
dc.contributor.author | Hernández González, Ignacio | - |
dc.contributor.author | Herrero Huertas, Julia | - |
dc.contributor.author | Jara Palomares, Luis | - |
dc.contributor.author | Jiménez Arjona, Josefa | - |
dc.contributor.author | Lázaro Salvador, María | - |
dc.contributor.author | López Ramón, Marta | - |
dc.contributor.author | López Reyes, Raquel | - |
dc.contributor.author | Mazo Etxaniz, Francisco Javier | - |
dc.contributor.author | Naranjo Velasco, Virginia | - |
dc.contributor.author | Otero Candelera, Remedios | - |
dc.contributor.author | Otero González, Isabel | - |
dc.contributor.author | Rodríguez Lozano, Beatriz | - |
dc.contributor.author | Rodríguez Nieto, María Jesús | - |
dc.contributor.author | Rueda Soriano, Joaquín | - |
dc.contributor.author | Safont, Belén | - |
dc.contributor.author | Sala Llinàs, Ernest | - |
dc.contributor.author | Sebastián, Laura | - |
dc.contributor.author | Segovia Cubero, Javier | - |
dc.contributor.author | Subirana Domenech, María Teresa | - |
dc.contributor.author | Baldà Masmiquel, Maria | - |
dc.contributor.author | Callejas Moraga, Eduardo | - |
dc.contributor.author | Chamorro, Antonio J. | - |
dc.contributor.author | Freire, Mayka | - |
dc.contributor.author | Herranz Marín, María Teresa | - |
dc.contributor.author | Marín Ballvé, Adela | - |
dc.contributor.author | Pestaña Fernández, Melany | - |
dc.contributor.author | Rodríguez Pintó, Ignasi | - |
dc.contributor.author | Salvador Cervelló, Gonzalo | - |
dc.contributor.author | Todolí Parra, José Antonio | - |
dc.contributor.author | Trapiella, Luis | - |
dc.contributor.author | Vargas Hitos, José Antonio | - |
dc.contributor.author | Rescle Consortium | - |
dc.contributor.author | Rehap Consortium | - |
dc.date.accessioned | 2022-04-25T10:10:21Z | - |
dc.date.available | 2022-04-25T10:10:21Z | - |
dc.date.issued | 2022-03-28 | - |
dc.identifier.issn | 2045-2322 | - |
dc.identifier.uri | http://hdl.handle.net/2445/185118 | - |
dc.description.abstract | 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. | - |
dc.format.extent | 11 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Springer Science and Business Media LLC | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1038/s41598-022-09353-z | - |
dc.relation.ispartof | Scientific Reports, 2022, vol. 12, num. 1 | - |
dc.relation.uri | https://doi.org/10.1038/s41598-022-09353-z | - |
dc.rights | cc by (c) Guillén del Castillo, Alfredo et al, 2022 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es/ | * |
dc.source | Articles publicats en revistes (Medicina) | - |
dc.subject.classification | Esclerodèrmia | - |
dc.subject.classification | Hipertensió pulmonar | - |
dc.subject.other | Scleroderma (Disease) | - |
dc.subject.other | Pulmonary hypertension | - |
dc.title | Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 733406 | - |
dc.date.updated | 2022-04-21T07:56:43Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 35347225 | - |
Appears in Collections: | Articles publicats en revistes (Medicina) Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
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733406.pdf | 1.21 MB | Adobe PDF | View/Open |
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