121031 20240319081004.0 doi 10.1038/s41598-022-09353-z sideral 130385 ART-2022-130385 eng Guillén-Del-Castillo, Alfredo Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension 2022 Access copy available to the general public Unrestricted 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. info:eu-repo/semantics/openAccess by http://creativecommons.org/licenses/by/3.0/es/ 4.6 2022 MULTIDISCIPLINARY SCIENCES 22 / 73 = 0.301 2022 Q2 T1 0.973 2022 Multidisciplinary 2022 Q1 7.5 2022 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Meseguer, Manuel López Fonollosa-Pla, Vicent Sáez Giménez, Berta Colunga-Argüelles, Dolores Revilla-López, Eva Rubio-Rivas, Manuel Ropero, Maria José Cristo Argibay, Ana Barberá, Joan Albert Pla Salas, Xavier Martínez Meñaca, Amaya Madroñero Vuelta, Ana Belén Lara Padrón, Antonio Sáez Comet, Luis Universidad de Zaragoza (orcid)0000-0002-9868-6498 Domingo Morera, Juan Antonio González-Echávarri, Cristina Mombiela, Teresa Ortego-Centeno, Norberto Marín González, Manuela Tolosa-Vilella, Carles Blanco, Isabel Escribano Subías, Pilar Simeón-Aznar, Carmen Pilar Aurtenetxe Pérez, Águeda Barberá, Joan Albert Barrios Garrido-Lestache, Elvira Bedate Díaz, Pedro Blanco, Isabel Cifrián, José Manuel Cristo Ropero, Maria Jose Domingo Morera, Juan Antonio Dos Subirá, Laura Elías Hernández, Teresa Escribano Subías, Pilar García Hernández, Francisco José Carbonell, Juan Gil Segovia, Ariadna González Valverde, Tamara Hermida Baldomero, Idaira Fámara Hernández Hernández-González, Ignacio Huertas, Julia Herrero Palomares, Luis Jara Arjona, Josefa Jiménez Padrón, Antonio Lara Lázaro-Salvador, María Meseguer, Manuel López López-Ramón, Marta López-Reyes, Raquel González, Manuela Marín Meñaca, Amaya Martínez Etxaniz, Francisco Javier Mazo Mombiela, Teresa Velasco, Virginia Naranjo Candelera, Remedios Otero González, Isabel Otero Revilla-López, Eva Lozano, Beatriz Rodríguez Nieto, María Jesús Rodríguez Soriano, Joaquín Rueda Giménez, Berta Sáez Safont, Belén Llinas, Ernest Sala Sebastián, Laura Cubero, Javier Segovia Domenech, María Teresa Subirana Argibay, Ana Masmiquel, Maria Baldà Moraga, Eduardo Callejas Chamorro, Antonio-J. Colunga-Argüelles, Dolores Fonollosa-Pla, Vicent Freire, Mayka González-Echávarri, Cristina Guillén-del-Castillo, Alfredo Marín, Maria Teresa Herranz Vuelta, Ana Belén Madroñero Ballvé, Adela Marín Universidad de Zaragoza (orcid)0000-0002-7815-8356 Ortego-Centeno, Norberto Fernández, Melany Pestaña Salas, Xavier Pla Pintó, Ignasi Rodríguez Rubio-Rivas, Manuel Comet, Luis Sáez Cervelló, Gonzalo Salvador Simeón-Aznar, Carmen Pilar Parra, José Antonio Todolí Tolosa-Vilella, Carles Trapiella, Luis Hitos, José Antonio Vargas Marín, Adela (REHAP Consortium) 1007 610 Universidad de Zaragoza Dpto. Medicina, Psiqu. y Derm. Area Medicina 12, 1 (2022), 5289 [11 PP.] Sci. rep. (Nat. Publ. Group) Scientific reports (Nature Publishing Group) 2045-2322 1241661 http://zaguan.unizar.es/record/121031/files/texto_completo.pdf Versión publicada 2582720 http://zaguan.unizar.es/record/121031/files/texto_completo.jpg?subformat=icon icon Versión publicada oai:zaguan.unizar.es:121031 articulos driver 2024-03-18-14:28:20 ARTICLE