RT Generic
T1 Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension (vol 12, 5289, 2022)
A1 Guillen-Del-Castillo, Alfredo
A1 Meseguer, Manuel Lopez
A1 Fonollosa-Pla, Vicent
A1 Gimenez, Berta Saez
A1 Colunga-Arguelles, Dolores
A1 Revilla-Lopez, Eva
A1 Rubio-Rivas, Manuel
A1 Ropero, Maria Jose Cristo
A1 Argibay, Ana
A1 Barbera, Joan Albert
A1 Salas, Xavier Pla
A1 Menaca, Amaya Martinez
A1 Vuelta, Ana Belen Madronero
A1 Padron, Antonio Lara
A1 Comet, Luis Saez
A1 Morera, Juan Antonio Domingo
A1 Gonzalez-Echavarri, Cristina
A1 Mombiela, Teresa
A1 Ortego-Centeno, Norberto
A1 Gonzalez, Manuela Marin
A1 Tolosa-Vilella, Carles
A1 Blanco, Isabel
A1 Subias, Pilar Escribano
A1 Simeon-Aznar, Carmen Pilar
A1 RESCLE Consortium, 
A1 REHAP Consortium, 
AB 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.
PB Nature Publishing Group
SN 2045-2322
YR 2022
FD 2022-03-11
LK http://hdl.handle.net/10668/19621
UL http://hdl.handle.net/10668/19621
LA en
NO Guillén-Del-Castillo A, Meseguer ML, Fonollosa-Pla V, Giménez BS, Colunga-Argüelles D, Revilla-López E, et al. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension. Sci Rep. 2022 Mar 28;12(1):5289. doi: 10.1038/s41598-022-09353-z. Erratum in: Sci Rep. 2022 Jul 28;12(1):12947.
DS RISalud
RD Apr 11, 2025