%0 Generic
%A Guillen-Del-Castillo, Alfredo
%A Meseguer, Manuel Lopez
%A Fonollosa-Pla, Vicent
%A Gimenez, Berta Saez
%A Colunga-Arguelles, Dolores
%A Revilla-Lopez, Eva
%A Rubio-Rivas, Manuel
%A Ropero, Maria Jose Cristo
%A Argibay, Ana
%A Barbera, Joan Albert
%A Salas, Xavier Pla
%A Menaca, Amaya Martinez
%A Vuelta, Ana Belen Madronero
%A Padron, Antonio Lara
%A Comet, Luis Saez
%A Morera, Juan Antonio Domingo
%A Gonzalez-Echavarri, Cristina
%A Mombiela, Teresa
%A Ortego-Centeno, Norberto
%A Gonzalez, Manuela Marin
%A Tolosa-Vilella, Carles
%A Blanco, Isabel
%A Subias, Pilar Escribano
%A Simeon-Aznar, Carmen Pilar
%A RESCLE Consortium
%A REHAP Consortium
%T Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension (vol 12, 5289, 2022)
%D 2022
%@ 2045-2322
%U http://hdl.handle.net/10668/19621
%X 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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