Publication: Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension (vol 12, 5289, 2022)
Loading...
Identifiers
Date
2022-03-11
Authors
Guillen-Del-Castillo, Alfredo
Meseguer, Manuel Lopez
Fonollosa-Pla, Vicent
Gimenez, Berta Saez
Colunga-Arguelles, Dolores
Revilla-Lopez, Eva
Rubio-Rivas, Manuel
Ropero, Maria Jose Cristo
Argibay, Ana
Barbera, Joan Albert
Advisors
Journal Title
Journal ISSN
Volume Title
Publisher
Nature Publishing Group
Abstract
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.
Description
MeSH Terms
DeCS Terms
CIE Terms
Keywords
Citation
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.