2026-04-14T05:16:04Zhttps://recercat.cat/oai/requestoai:recercat.cat:11351/79992025-10-24T10:39:34Zcom_2072_378070com_2072_378040col_2072_378092
RECERCAT
author
Guillen Del Castillo, Alfredo
author
Fonollosa Pla, Vicenç
author
Saez Gimenez, Berta
author
Colunga‑Argüelles, Dolores
author
Revilla Lopez, Eva Maria
author
Simeón Aznar, Carmen Pilar
author
López Meseguer, Manuel
2025-10-24T10:39:34Z
2025-10-24T10:39:34Z
2022-08-12T09:52:41Z2022-08-12T09:52:41Z2022-03-28
http://hdl.handle.net/11351/7999
Respiratory tract diseases; Systemic sclerosisMalalties del tracte respiratori; Esclerosi sistèmicaEnfermedades del tracto respiratorio; Esclerosis sistémicaTo 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.
Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess
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
Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion