doi:10.1038/s41598-022-09353-zengGuillén-Del-Castillo, AlfredoMeseguer, Manuel LópezFonollosa-Pla, VicentSáez Giménez, BertaColunga-Argüelles, DoloresRevilla-López, EvaRubio-Rivas, ManuelRopero, Maria José CristoArgibay, AnaBarberá, Joan AlbertPla Salas, XavierMartínez Meñaca, AmayaMadroñero Vuelta, Ana BelénLara Padrón, AntonioSáez Comet, LuisDomingo Morera, Juan AntonioGonzález-Echávarri, CristinaMombiela, TeresaOrtego-Centeno, NorbertoMarín González, ManuelaTolosa-Vilella, CarlesBlanco, IsabelEscribano Subías, PilarSimeón-Aznar, Carmen PilarAurtenetxe Pérez, ÁguedaBarberá, Joan AlbertBarrios Garrido-Lestache, ElviraBedate Díaz, PedroBlanco, IsabelCifrián, José ManuelCristo Ropero, Maria JoseDomingo Morera, Juan AntonioDos Subirá, LauraElías Hernández, TeresaEscribano Subías, PilarGarcía Hernández, Francisco JoséCarbonell, Juan GilSegovia, Ariadna GonzálezValverde, Tamara HermidaBaldomero, Idaira Fámara HernándezHernández-González, IgnacioHuertas, Julia HerreroPalomares, Luis JaraArjona, Josefa JiménezPadrón, Antonio LaraLázaro-Salvador, MaríaMeseguer, Manuel LópezLópez-Ramón, MartaLópez-Reyes, RaquelGonzález, Manuela MarínMeñaca, Amaya MartínezEtxaniz, Francisco Javier MazoMombiela, TeresaVelasco, Virginia NaranjoCandelera, Remedios OteroGonzález, Isabel OteroRevilla-López, EvaLozano, Beatriz RodríguezNieto, María Jesús RodríguezSoriano, Joaquín RuedaGiménez, Berta SáezSafont, BelénLlinas, Ernest SalaSebastián, LauraCubero, Javier SegoviaDomenech, María Teresa SubiranaArgibay, AnaMasmiquel, Maria BaldàMoraga, Eduardo CallejasChamorro, Antonio-J.Colunga-Argüelles, DoloresFonollosa-Pla, VicentFreire, MaykaGonzález-Echávarri, CristinaGuillén-del-Castillo, AlfredoMarín, Maria Teresa HerranzVuelta, Ana Belén MadroñeroBallvé, Adela MarínOrtego-Centeno, NorbertoFernández, Melany PestañaSalas, Xavier PlaPintó, Ignasi RodríguezRubio-Rivas, ManuelComet, Luis SáezCervelló, Gonzalo SalvadorSimeón-Aznar, Carmen PilarParra, José Antonio TodolíTolosa-Vilella, CarlesTrapiella, LuisHitos, José Antonio VargasMarín, Adela (REHAP Consortium)Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertensionART-2022-130385To 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.2022http://zaguan.unizar.es/record/12103110.1038/s41598-022-09353-zhttp://zaguan.unizar.es/record/121031oai:zaguan.unizar.es:121031Scientific reports (Nature Publishing Group) 12, 1 (2022), 5289 [11 PP.]byhttp://creativecommons.org/licenses/by/3.0/es/info:eu-repo/semantics/openAccess