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