Pulmonary hypertension in Spanish patients with systemic sclerosis. Data from the RESCLE registry

Fecha de publicación:

Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Garcia-Hernandez, FJ
  • Castillo-Palma, MJ
  • Tolosa-Vilella, C
  • Guillen-del Castillo, A
  • Rubio-Rivas, M
  • Freire, M
  • Vargas-Hitos, JA
  • Rodriguez-Carballeira, M
  • Espinosa-Garriga, G
  • Colunga-Arguelles, D
  • Ortego-Centeno, N
  • Trapiella-Martinez, L
  • Rodero-Roldan, MM
  • Pla-Salas, X
  • Perales-Fraile, I
  • del Campo, IPM
  • Chamorro, AJ
  • Gimenez, RAFD
  • Madronero-Vuelta, AB
  • Ruiz-Munoz, M
  • Fonollosa-Pla, V
  • Simeon-Aznar, CP
  • SSSG
  • Autoimmune Dis Study Grp GEAS
  • Spanish Soc Internal Med SEMI

Grupos

Abstract

IntroductionOur objective was to evaluate the pulmonary hypertension (PH) data for Spanish patients with systemic sclerosis (SSc), define the PH types and determine the associated factors.MethodDescriptive study of PH-related data from the multicentre RESCLE registry. Estimated systolic pulmonary artery pressure (esPAP), measured via echocardiogram was considered elevated if 35mmHg. Left heart disease (LHD) and interstitial lung disease (ILD) were identified. When performed, data from right heart catheterisation (RHC) were collected.ResultsesPAP was elevated in 350 of 808 patients (43.3%). One hundred and forty-four patients (17.8%) were considered to have PH (88 via RHC and the rest due to elevated esPAP along with evidence of significant LHD or ILD): PAH 3.7%, secondary to ILD 8.3%, secondary to LHD 2.8% and unclassified 3%. Prevalence of elevated esPAP was greater in diffuse SSc (dSSc) than in limited scleroderma (lSSc) (50.5 vs. 42.2%, p 0.046). In the group with elevated esPAP, a lower prevalence of anti-centromere antibodies (41.9% vs. 52.3%, p 0.006) and a greater prevalence of anti-topoisomerase-1 antibodies (ATA) (25.1% vs. 18.6%, p 0.04) were observed compared to the group with normal esPAP. Patients with elevated esPAP had a lower rate of digital ulcers (50.6% vs. 60.2%, p 0.007) and esophageal involvement (83.6% vs. 88.7%, p 0.07) and higher rate of renal crisis (4.6% vs. 1.8%, p 0.066).ConclusionsPrevalence of PAH was lower than expected (3.7%). Probability of having elevated esPAP was higher among patients with dSSc and among those with ATA.

Datos de la publicación

ISSN/ISSNe:
0770-3198, 1434-9949

CLINICAL RHEUMATOLOGY  SPRINGER LONDON LTD

Tipo:
Article
Páginas:
1117-1124
PubMed:
30535994
Factor de Impacto:
0,755 SCImago
Cuartil:
Q2 SCImago

Citas Recibidas en Web of Science: 8

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Keywords

  • Anti-centromere antibodies; Pulmonary hypertension; Systemic sclerosis

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