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

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 Span...

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Detalles Bibliográficos
Autores: Guillén-Del-Castillo A, Meseguer ML, Fonollosa-Pla V, Giménez BS, Colunga-Argüelles D, Revilla-López E, Rubio-Rivas M, Ropero MJC, Argibay A, Barberá JA, Salas XP, Meñaca AM, Vuelta ABM, Padrón AL, Comet LS, Morera JAD, González-Echávarri C, Mombiela T, Ortego-Centeno N, González MM, Tolosa-Vilella C, Blanco I, Subías PE, Simeón-Aznar CP, RESCLE Consortium, REHAP Consortium
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:España
Institución:Institut d'Investigació i Innovació Parc Taulí (I3PT)
Repositorio:r-I3PT. Repositorio Institucional Producción Científica del Institut d'Investigació i Innovació Parc Taulí
OAI Identifier:oai:i3pt.fundanetsuite.com:p1598
Acceso en línea:https://i3pt.portalinvestigacion.com/publicaciones/1598
https://www.scopus.com/inward/record.uri?eid=2-s2.0-85127250841&doi=10.1038%2fs41598-022-09353-z&partnerID=40&md5=7c5b8193a5d353ae3030ca032a238110
Access Level:acceso abierto
Palabra clave:Familial Primary Pulmonary Hypertension
Humans
Hypertension, Pulmonary
Lung Diseases, Interstitial
Pulmonary Arterial Hypertension
Scleroderma, Systemic
human
interstitial lung disease
pulmonary hypertension
systemic sclerosis
Descripción
Sumario: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.

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