Guillen-Del-Castillo, AlfredoMeseguer, Manuel LopezFonollosa-Pla, VicentGimenez, Berta SaezColunga-Arguelles, DoloresRevilla-Lopez, EvaRubio-Rivas, ManuelRopero, Maria Jose CristoArgibay, AnaBarbera, Joan AlbertSalas, Xavier PlaMenaca, Amaya MartinezVuelta, Ana Belen MadroneroPadron, Antonio LaraComet, Luis SaezMorera, Juan Antonio DomingoGonzalez-Echavarri, CristinaMombiela, TeresaOrtego-Centeno, NorbertoGonzalez, Manuela MarinTolosa-Vilella, CarlesBlanco, IsabelSubias, Pilar EscribanoSimeon-Aznar, Carmen PilarRESCLE ConsortiumREHAP Consortium2023-05-032023-05-032022-03-11Guillén-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):12947.2045-2322http://hdl.handle.net/10668/19621To 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.enAttribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/corrigendumopen access10.1038/s41598-022-09353-zhttps://www.nature.com/articles/s41598-022-17525-0.pdf833071900087