Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
Authors: Guillén-Del-Castillo Alfredo , Meseguer Manuel López , Fonollosa-Pla Vicent , Giménez Berta Sáez , Colunga-Argüelles Dolores , Revilla-López Eva , Rubio-Rivas Manuel , Ropero Maria Jose Cristo , Argibay Ana , Barberá Joan Albert , Salas Xavier Pla , Meñaca Amaya Martínez , Vuelta Ana Belén Madroñero , Padrón Antonio Lara , Comet Luis Sáez , Morera Juan Antonio Domingo , González-Echávarri Cristina , Mombiela Teresa , Ortego-Centeno Norberto , González Manuela Marín , Tolosa-Vilella Carles , Blanco Isabel , Subías Pilar Escribano , Simeón-Aznar Carmen Pilar ,   RESCLE Consortium, Pérez Águeda Aurtenetxe , Barberá Joan Albert , Garrido-Lestache Elvira Barrios , Díaz Pedro Bedate , Blanco Isabel , Cifrián José Manuel , Ropero Maria Jose Cristo , Morera Juan Antonio Domingo , Dos Subirá Laura , Hernández Teresa Elías , Subías Pilar Escribano , Hernández Francisco José García , 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 ,   REHAP Consortium, 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 , 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 
Published: March 2022

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.