000121031 001__ 121031
000121031 005__ 20240319081004.0
000121031 0247_ $$2doi$$a10.1038/s41598-022-09353-z
000121031 0248_ $$2sideral$$a130385
000121031 037__ $$aART-2022-130385
000121031 041__ $$aeng
000121031 100__ $$aGuillén-Del-Castillo, Alfredo
000121031 245__ $$aImpact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
000121031 260__ $$c2022
000121031 5060_ $$aAccess copy available to the general public$$fUnrestricted
000121031 5203_ $$aTo 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.
000121031 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/
000121031 590__ $$a4.6$$b2022
000121031 592__ $$a0.973$$b2022
000121031 591__ $$aMULTIDISCIPLINARY SCIENCES$$b22 / 73 = 0.301$$c2022$$dQ2$$eT1
000121031 593__ $$aMultidisciplinary$$c2022$$dQ1
000121031 594__ $$a7.5$$b2022
000121031 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000121031 700__ $$aMeseguer, Manuel López
000121031 700__ $$aFonollosa-Pla, Vicent
000121031 700__ $$aSáez Giménez, Berta
000121031 700__ $$aColunga-Argüelles, Dolores
000121031 700__ $$aRevilla-López, Eva
000121031 700__ $$aRubio-Rivas, Manuel
000121031 700__ $$aRopero, Maria José Cristo
000121031 700__ $$aArgibay, Ana
000121031 700__ $$aBarberá, Joan Albert
000121031 700__ $$aPla Salas, Xavier
000121031 700__ $$aMartínez Meñaca, Amaya
000121031 700__ $$aMadroñero Vuelta, Ana Belén
000121031 700__ $$aLara Padrón, Antonio
000121031 700__ $$0(orcid)0000-0002-9868-6498$$aSáez Comet, Luis$$uUniversidad de Zaragoza
000121031 700__ $$aDomingo Morera, Juan Antonio
000121031 700__ $$aGonzález-Echávarri, Cristina
000121031 700__ $$aMombiela, Teresa
000121031 700__ $$aOrtego-Centeno, Norberto
000121031 700__ $$aMarín González, Manuela
000121031 700__ $$aTolosa-Vilella, Carles
000121031 700__ $$aBlanco, Isabel
000121031 700__ $$aEscribano Subías, Pilar
000121031 700__ $$aSimeón-Aznar, Carmen Pilar
000121031 700__ $$aAurtenetxe Pérez, Águeda
000121031 700__ $$aBarberá, Joan Albert
000121031 700__ $$aBarrios Garrido-Lestache, Elvira
000121031 700__ $$aBedate Díaz, Pedro
000121031 700__ $$aBlanco, Isabel
000121031 700__ $$aCifrián, José Manuel
000121031 700__ $$aCristo Ropero, Maria Jose
000121031 700__ $$aDomingo Morera, Juan Antonio
000121031 700__ $$aDos Subirá, Laura
000121031 700__ $$aElías Hernández, Teresa
000121031 700__ $$aEscribano Subías, Pilar
000121031 700__ $$aGarcía Hernández, Francisco José
000121031 700__ $$aCarbonell, Juan Gil
000121031 700__ $$aSegovia, Ariadna González
000121031 700__ $$aValverde, Tamara Hermida
000121031 700__ $$aBaldomero, Idaira Fámara Hernández
000121031 700__ $$aHernández-González, Ignacio
000121031 700__ $$aHuertas, Julia Herrero
000121031 700__ $$aPalomares, Luis Jara
000121031 700__ $$aArjona, Josefa Jiménez
000121031 700__ $$aPadrón, Antonio Lara
000121031 700__ $$aLázaro-Salvador, María
000121031 700__ $$aMeseguer, Manuel López
000121031 700__ $$aLópez-Ramón, Marta
000121031 700__ $$aLópez-Reyes, Raquel
000121031 700__ $$aGonzález, Manuela Marín
000121031 700__ $$aMeñaca, Amaya Martínez
000121031 700__ $$aEtxaniz, Francisco Javier Mazo
000121031 700__ $$aMombiela, Teresa
000121031 700__ $$aVelasco, Virginia Naranjo
000121031 700__ $$aCandelera, Remedios Otero
000121031 700__ $$aGonzález, Isabel Otero
000121031 700__ $$aRevilla-López, Eva
000121031 700__ $$aLozano, Beatriz Rodríguez
000121031 700__ $$aNieto, María Jesús Rodríguez
000121031 700__ $$aSoriano, Joaquín Rueda
000121031 700__ $$aGiménez, Berta Sáez
000121031 700__ $$aSafont, Belén
000121031 700__ $$aLlinas, Ernest Sala
000121031 700__ $$aSebastián, Laura
000121031 700__ $$aCubero, Javier Segovia
000121031 700__ $$aDomenech, María Teresa Subirana
000121031 700__ $$aArgibay, Ana
000121031 700__ $$aMasmiquel, Maria Baldà
000121031 700__ $$aMoraga, Eduardo Callejas
000121031 700__ $$aChamorro, Antonio-J.
000121031 700__ $$aColunga-Argüelles, Dolores
000121031 700__ $$aFonollosa-Pla, Vicent
000121031 700__ $$aFreire, Mayka
000121031 700__ $$aGonzález-Echávarri, Cristina
000121031 700__ $$aGuillén-del-Castillo, Alfredo
000121031 700__ $$aMarín, Maria Teresa Herranz
000121031 700__ $$aVuelta, Ana Belén Madroñero
000121031 700__ $$0(orcid)0000-0002-7815-8356$$aBallvé, Adela Marín$$uUniversidad de Zaragoza
000121031 700__ $$aOrtego-Centeno, Norberto
000121031 700__ $$aFernández, Melany Pestaña
000121031 700__ $$aSalas, Xavier Pla
000121031 700__ $$aPintó, Ignasi Rodríguez
000121031 700__ $$aRubio-Rivas, Manuel
000121031 700__ $$aComet, Luis Sáez
000121031 700__ $$aCervelló, Gonzalo Salvador
000121031 700__ $$aSimeón-Aznar, Carmen Pilar
000121031 700__ $$aParra, José Antonio Todolí
000121031 700__ $$aTolosa-Vilella, Carles
000121031 700__ $$aTrapiella, Luis
000121031 700__ $$aHitos, José Antonio Vargas
000121031 700__ $$aMarín, Adela (REHAP Consortium)
000121031 7102_ $$11007$$2610$$aUniversidad de Zaragoza$$bDpto. Medicina, Psiqu. y Derm.$$cArea Medicina
000121031 773__ $$g12, 1 (2022), 5289 [11 PP.]$$pSci. rep. (Nat. Publ. Group)$$tScientific reports (Nature Publishing Group)$$x2045-2322
000121031 8564_ $$s1241661$$uhttps://zaguan.unizar.es/record/121031/files/texto_completo.pdf$$yVersión publicada
000121031 8564_ $$s2582720$$uhttps://zaguan.unizar.es/record/121031/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000121031 909CO $$ooai:zaguan.unizar.es:121031$$particulos$$pdriver
000121031 951__ $$a2024-03-18-14:28:20
000121031 980__ $$aARTICLE