2026-04-18T00:06:38Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/79992025-10-24T10:39:34Zcom_2072_378070com_2072_378040col_2072_378092

Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension Guillen Del Castillo, Alfredo Fonollosa Pla, Vicenç Saez Gimenez, Berta Colunga‑Argüelles, Dolores Revilla Lopez, Eva Maria Simeón Aznar, Carmen Pilar López Meseguer, Manuel Esclerosi sistemàtica progressiva - Tractament Hipotensió arterial Pulmons - Malalties DISEASES::Respiratory Tract Diseases::Lung Diseases::Lung Diseases, Interstitial DISEASES::Respiratory Tract Diseases::Lung Diseases::Hypertension, Pulmonary DISEASES::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::enfermedades pulmonares intersticiales ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::hipertensión pulmonar ENFERMEDADES::enfermedades de la piel y tejido conjuntivo::enfermedades del tejido conjuntivo::esclerodermia sistémica Respiratory tract diseases; Systemic sclerosis Malalties del tracte respiratori; Esclerosi sistèmica Enfermedades del tracto respiratorio; Esclerosis sistémica 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. 2025-10-24T10:39:34Z 2025-10-24T10:39:34Z 2025-10-24T10:39:34Z 2022-08-12T09:52:41Z 2022-08-12T09:52:41Z 2022-03-28 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/7999 Scientific Reports;12 https://doi.org/10.1038/s41598-022-09353-z Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess Nature Research Scientia