Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

Guillén-Del-Castillo, Alfredo ; Meseguer, Manuel López ; Fonollosa-Pla, Vicent ; Sáez Giménez, Berta ; Colunga-Argüelles, Dolores ; Revilla-López, Eva ; Rubio-Rivas, Manuel ; Ropero, Maria José Cristo ; Argibay, Ana ; Barberá, Joan Albert ; Pla Salas, Xavier ; Martínez Meñaca, Amaya ; Madroñero Vuelta, Ana Belén ; Lara Padrón, Antonio ; Sáez Comet, Luis (Universidad de Zaragoza) ; Domingo Morera, Juan Antonio ; González-Echávarri, Cristina ; Mombiela, Teresa ; Ortego-Centeno, Norberto ; Marín González, Manuela ; Tolosa-Vilella, Carles ; Blanco, Isabel ; Escribano Subías, Pilar ; Simeón-Aznar, Carmen Pilar ; Aurtenetxe Pérez, Águeda ; Barberá, Joan Albert ; Barrios Garrido-Lestache, Elvira ; Bedate Díaz, Pedro ; Blanco, Isabel ; Cifrián, José Manuel ; Cristo Ropero, Maria Jose ; Domingo Morera, Juan Antonio ; Dos Subirá, Laura ; Elías Hernández, Teresa ; Escribano Subías, Pilar ; García Hernández, Francisco José ; Carbonell, Juan Gil ; Segovia, Ariadna González ; Valverde, Tamara Hermida ; Baldomero, Idaira Fámara Hernández ; Hernández-González, Ignacio ; Huertas, Julia Herrero ; Palomares, Luis Jara ; Arjona, Josefa Jiménez ; Padrón, Antonio Lara ; Lázaro-Salvador, María ; Meseguer, Manuel López ; López-Ramón, Marta ; López-Reyes, Raquel ; González, Manuela Marín ; Meñaca, Amaya Martínez ; Etxaniz, Francisco Javier Mazo ; Mombiela, Teresa ; Velasco, Virginia Naranjo ; Candelera, Remedios Otero ; González, Isabel Otero ; Revilla-López, Eva ; Lozano, Beatriz Rodríguez ; Nieto, María Jesús Rodríguez ; Soriano, Joaquín Rueda ; Giménez, Berta Sáez ; Safont, Belén ; Llinas, Ernest Sala ; Sebastián, Laura ; Cubero, Javier Segovia ; Domenech, María Teresa Subirana ; Argibay, Ana ; Masmiquel, Maria Baldà ; Moraga, Eduardo Callejas ; Chamorro, Antonio-J. ; Colunga-Argüelles, Dolores ; Fonollosa-Pla, Vicent ; Freire, Mayka ; González-Echávarri, Cristina ; Guillén-del-Castillo, Alfredo ; Marín, Maria Teresa Herranz ; Vuelta, Ana Belén Madroñero ; Ballvé, Adela Marín (Universidad de Zaragoza) ; Ortego-Centeno, Norberto ; Fernández, Melany Pestaña ; Salas, Xavier Pla ; Pintó, Ignasi Rodríguez ; Rubio-Rivas, Manuel ; Comet, Luis Sáez ; Cervelló, Gonzalo Salvador ; Simeón-Aznar, Carmen Pilar ; Parra, José Antonio Todolí ; Tolosa-Vilella, Carles ; Trapiella, Luis ; Hitos, José Antonio Vargas ; Marín, Adela (REHAP Consortium)

Resumen: 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.

Idioma: Inglés

DOI: 10.1038/s41598-022-09353-z

Año: 2022

Publicado en: Scientific reports (Nature Publishing Group) 12, 1 (2022), 5289 [11 PP.]
ISSN: 2045-2322

Factor impacto JCR: 4.6 (2022)

Categ. JCR: MULTIDISCIPLINARY SCIENCES rank: 22 / 73 = 0.301 (2022) - Q2 - T1

Factor impacto CITESCORE: 7.5 - General (Q1)

Factor impacto SCIMAGO: 0.973 - Multidisciplinary (Q1)

Tipo y forma: Article (Published version)

Área (Departamento): Area Medicina (Dpto. Medicina, Psiqu. y Derm.)