Portal de investigación
Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
Fecha de publicación:
Autores de I3PT
Participantes ajenos a I3PT
- 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
- Blanco I
- Subías PE
- Simeón-Aznar CP
- RESCLE Consortium
- REHAP Consortium
Grupos de Investigación
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.
Datos de la publicación
- ISSN/ISSNe:
- 2045-2322, 2045-2322
- Tipo:
- Article
- Páginas:
- 5289-5289
- PubMed:
- 35347225
- Enlace a otro recurso:
- www.scopus.com
Scientific Reports NATURE PORTFOLIO
Citas Recibidas en Web of Science: 17
Citas Recibidas en Scopus: 2
Documentos
- No hay documentos
Filiaciones
Keywords
- Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Pulmonary Arterial Hypertension; Scleroderma, Systemic; human; interstitial lung disease; pulmonary hypertension; systemic sclerosis
Campos de Estudio
Cita
Guillén A,Meseguer ML,Fonollosa V,Giménez BS,Colunga D,Revilla E,Rubio M,Ropero MJC,Argibay A,Barberá JA et al. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension. Sci Rep. 2022. 12. (1):p. 5289-5289. DOI: 10.1038/s41598-022-09353-z.
Guillén A,Meseguer ML,Fonollosa V,Giménez BS,Colunga D,Revilla E,Rubio M,Ropero MJC,Argibay A,Barberá JA,Salas XP,Meñaca AM,Vuelta A,Padrón AL,Comet LS,Morera J,González C,Mombiela T,Ortego N,González MM,Tolosa C,Blanco I,Subías PE,Simeón CP,RESCLE C,REHAP C. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension. Sci Rep. 2022. 12. (1):p. 5289-5289. IF:4,600. (2).