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Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/185118
Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
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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.
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GUILLÉN DEL CASTILLO, Alfredo, LÓPEZ MESEGUER, Manuel, FONOLLOSA PLA, Vicent, SÁEZ GIMÉNEZ, Berta, COLUNGA ARGÜELLES, Dolores, REVILLA LÓPEZ, Eva, RUBIO RIVAS, Manuel, CRISTO ROPERO, Maria jose, ARGIBAY, Ana, BARBERÀ I MIR, Joan albert, PLA SALAS, Xavier, MARTÍNEZ MEÑACA, Amaya, MADROÑERO VUELTA, Ana belén, LARA PADRÓN, Antonio, SÁEZ COMET, Luis, DOMINGO MORERA, Juan antonio, GONZÁLEZ ECHÁVARRI, Cristina, MOMBIELA, Teresa, ORTEGO CENTENO, Norberto, MARÍN GONZÁLEZ, Manuela, TOLOSA VILELLA, Carles, BLANCO VICH, Isabel, ESCRIBANO SUBIAS, Pilar, SIMEÓN AZNAR, Carmen pilar, AURTENETXE PÉREZ, Águeda, BARBERÀ I MIR, Joan albert, BARRIOS GARRIDO-LESTACHE, Elvira, BEDATE DÍAZ, Pedro, CIFRIÁN, José manuel, DOS SUBIRÁ, Laura, HERNÁNDEZ, Teresa elías, GARCÍA HERNÁNDEZ, Francisco josé, GIL CARBONELL, Juan, GONZÁLEZ SEGOVIA, Ariadna, HERMIDA VALVERDE, Tamara, HERNÁNDEZ BALDOMERO, Idaira fámara, HERNÁNDEZ GONZÁLEZ, Ignacio, HERRERO HUERTAS, Julia, JARA PALOMARES, Luis, JIMÉNEZ ARJONA, Josefa, LÁZARO SALVADOR, María, LÓPEZ RAMÓN, Marta, LÓPEZ REYES, Raquel, MAZO ETXANIZ, Francisco javier, NARANJO VELASCO, Virginia, OTERO CANDELERA, Remedios, OTERO GONZÁLEZ, Isabel, RODRÍGUEZ LOZANO, Beatriz, RODRÍGUEZ NIETO, María jesús, RUEDA SORIANO, Joaquín, SAFONT, Belén, SALA LLINÀS, Ernest, SEBASTIÁN, Laura, SEGOVIA CUBERO, Javier, SUBIRANA DOMENECH, María teresa, BALDÀ MASMIQUEL, Maria, CALLEJAS MORAGA, Eduardo, CHAMORRO, Antonio j., FREIRE, Mayka, HERRANZ MARÍN, María teresa, MARÍN BALLVÉ, Adela, PESTAÑA FERNÁNDEZ, Melany, RODRÍGUEZ PINTÓ, Ignasi, SALVADOR CERVELLÓ, Gonzalo, TODOLÍ PARRA, José antonio, TRAPIELLA, Luis, VARGAS HITOS, José antonio, Rescle Consortium, Rehap Consortium. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension. _Scientific Reports_. 2022. Vol. 12, núm. 1. [consulta: 19 de abril de 2026]. ISSN: 2045-2322. [Disponible a: https://hdl.handle.net/2445/185118]