Author Correction : Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
Guillén-Del-Castillo, Alfredo (Hospital Universitari Vall d'Hebron)
López-Meseguer, Manuel (Hospital Universitari Vall d'Hebron)
Fonollosa Pla, Vicent (Hospital Universitari Vall d'Hebron)
Sáez-Giménez, Berta (Hospital Universitari Vall d'Hebron)
Colunga-Argüelles, Dolores (Hospital Universitario Central de Asturias)
Revilla-López, Eva (Hospital Universitari Vall d'Hebron)
Rubio-Rivas, Manuel (Hospital Universitari de Bellvitge)
Ropero, María José Cristo (Hospital Universitario 12 de Octubre (Madrid))
Argibay, Ana (Complexo Hospitalario Universitario de Vigo)
Barberà i Mir, Joan Albert (Hospital Clínic i Provincial de Barcelona)
Salas, Xavier Pla (Consorci Hospitalari de Vic)
Meñaca, Amaya Martínez (Hospital Universitario Marqués de Valdecilla (Santander, Cantabria))
Vuelta, Ana Belén Madroñero (Hospital General Universitario San Jorge (Osca, Aragó))
Padrón, Antonio Lara (Hospital Universitario de Canarias (La Laguna))
Comet, Luis Sáez (Hospital Universitario Miguel Servet (Saragossa))
Morera, Juan Antonio Domingo (Hospital Universitario Miguel Servet (Saragossa))
González-Echávarri, Cristina (Hospital Universitario de Cruces (Barakaldo, País Basc))
Mombiela, Teresa (Hospital General Universitario Gregorio Marañón)
Ortego-Centeno, Norberto (Hospital Universitario San Cecilio (Granada))
González, Manuela Marín (Hospital Clínic Universitari (València))
Tolosa-Vilella, Carles (Parc Taulí Hospital Universitari. Institut d'Investigació i Innovació Parc Taulí (I3PT))
Blanco, Isabel (Hospital Clínic i Provincial de Barcelona)
Subías, Pilar Escribano (Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares)
Simeón-Aznar, Carmen Pilar (Hospital Universitari Vall d'Hebron)

Date:	2022
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.
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	Systemic sclerosis ; Respiratory tract diseases
Published in:	Scientific reports, Vol. 12 (july 2022) , ISSN 2045-2322

Article original: https://ddd.uab.cat/record/258149?ln=es
Author Correction: https://ddd.uab.cat/record/282047?ln=es
DOI: 10.1038/s41598-022-17525-0
PMID: 35902683

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