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

Fecha de publicación:

Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Guillén-Del-Castillo A.
  • Meseguer M.L.
  • Fonollosa-Pla V.
  • Giménez B.S.
  • Colunga-Argüelles D.
  • Revilla-López E.
  • Rubio-Rivas M.
  • Ropero M.J.C.
  • Argibay A.
  • Mir J.A.B.
  • Salas X.P.
  • Meñaca A.M.
  • Vuelta A.B.M.
  • Padrón A.L.
  • Comet L.S.
  • Morera J.A.D.
  • Mombiela T.
  • Ortego-Centeno N.
  • González M.M.
  • Tolosa-Vilella C.
  • Blanco I.
  • Subías P.E.
  • Simeón-Aznar C.P.
  • Pérez Á.A.
  • Barberá J.A.
  • Garrido-Lestache E.B.
  • Díaz P.B.
  • Cifrián J.M.
  • Dos Subirá L.
  • Hernández T.E.
  • Hernández F.J.G.
  • Carbonell J.G.
  • Segovia A.G.
  • Valverde T.H.
  • Baldomero I.F.H.
  • Hernández-González I.
  • Huertas J.H.
  • Palomares L.J.
  • Arjona J.J.
  • Lázaro-Salvador M.
  • López-Ramón M.
  • Etxaniz F.J.M.
  • Velasco V.N.
  • Candelera R.O.
  • González I.O.
  • Lozano B.R.
  • Nieto M.J.R.
  • Safont B.
  • Llinas E.S.
  • Sebastián L.
  • Cubero J.S.
  • Domenech M.T.S.
  • Masmiquel M.B.
  • Moraga E.C.
  • Chamorro A.-J.
  • Freire M.
  • González-Echávarri C.
  • Marín M.T.H.
  • Ballvé A.M.
  • Todoli
  • Fernández M.P.
  • Pintó I.R.
  • Cervelló G.S.
  • Trapiella L.
  • Hitos J.A.V.

Grupos

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. © 2022, The Author(s).

Datos de la publicación

ISSN/ISSNe:
2045-2322, 2045-2322

Scientific Reports  NATURE PORTFOLIO

Tipo:
Article
Páginas:
-
PubMed:
35347225
Enlace a otro recurso:
www.scopus.com
Factor de Impacto:
1,005 SCImago ℠
Cuartil:
Q1 SCImago ℠

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Keywords

  • Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Pulmonary Arterial Hypertension; Scleroderma, Systemic; human; interstitial lung disease; pulmonary hypertension; systemic sclerosis

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