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Porto-sinusoidal vascular liver disorder with portal hypertension: Natural history and long-term outcome

Fecha de publicación:

Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Magaz, M
  • Giudicelli-Lett, H
  • Abraldes, JG
  • Nicoara-Farcau, O
  • Turon, F
  • Rajoriya, N
  • Goel, A
  • Raymenants, K
  • Hillaire, S
  • Téllez, L
  • Elkrief, L
  • Procopet, B
  • Orts, L
  • Nery, F
  • Shukla, A
  • Larrue, H
  • Degroote, H
  • Llop, E
  • Turco, L
  • Indulti, F
  • Gioia, S
  • Tosetti, G
  • Bitto, N
  • Becchetti, C
  • Alvarado, E
  • Roig, C
  • Diaz, R
  • Praktiknjo, M
  • Konicek, AL
  • Olivas, P
  • Fortea, JI
  • Masnou, H
  • Puente, A
  • Ardèvol, A
  • Navascués, CA
  • Romero-Gutiérrez, M
  • Scheiner, B
  • Semmler, G
  • Mandorfer, M
  • Damiao, F
  • Baiges, A
  • Ojeda, A
  • Simón-Talero, M
  • González-Alayón, C
  • Díaz, A
  • García-Criado, A
  • De Gottardi, A
  • Hernández-Guerra, M
  • Genescà, J
  • Drilhon, N
  • Ferreira, CN
  • Reiberger, T
  • Rodríguez, M
  • Morillas, RM
  • Crespo, J
  • Trebicka, J
  • Bañares, R
  • Villanueva, C
  • Berzigotti, A
  • Primignani, M
  • La Mura, V
  • Riggio, O
  • Schepis, F
  • Verhelst, X
  • Calleja, JL
  • Bureau, C
  • Albillos, A
  • Nevens, F
  • Hernández-Gea, V
  • Tripathi, D
  • Rautou, PE
  • García-Pagán, JC
  • ERN RARE-LIVER

Grupos

Abstract

Background & Aims: Current knowledge of the natural history of patients with porto-sinusoidal vascular disorder (PSVD) is derived from small studies. The aim of the present study was to determine the natural history of PSVD and prognostic factors in a large multicenter cohort of patients. Methods: We performed a retrospective study on patients with PSVD and signs of portal hypertension (PH) prospectively registered in 27 centers. Results: A total of 587 patients were included, median age of 47 years and 38% were women. Four-hundred and one patients had an associated condition, which was graded as severe in 157. Median follow-up was 68 months. At diagnosis, 64% of patients were asymptomatic while 36% had a PH-related complication: PH-related bleeding in 112 patients, ascites in 117, and hepatic encephalopathy in 11. In those not presenting with bleeding, the incidence of first bleeding was 15% at 5 years, with a 5-year rebleeding rate of 18%. The 5-year cumulative incidence of new or worsening ascites was 18% and of developing portal vein thrombosis was 16%. Fifty (8.5%) patients received a liver transplantation and 109 (19%) died, including 55 non-liver-related deaths. Transplant-free survival was 97% and 83% at 1 and 5 years, respectively. Variables independently associated with transplant-free survival were age, ascites, serum bilirubin, albumin and creatinine levels at diagnosis and severe associated conditions. This allowed for the creation of a nomogram that accurately predicted prognosis. Conclusions: The prognosis of PSVD is strongly determined by the severity of the associated underlying conditions and parameters of liver and renal function. (c) 2024 European Association for the Study of the Liver. Published by Elsevier B.V. All rights are reserved, including those for text and data mining, AI training, and similar technologies.

Datos de la publicación

ISSN/ISSNe:
0168-8278, 1600-0641

JOURNAL OF HEPATOLOGY  ELSEVIER

Tipo:
Article
Páginas:
72-83
PubMed:
39181213
Enlace a otro recurso:
www.scopus.com
Factor de Impacto:
7,351 SCImago ℠
Cuartil:
Q1 SCImago ℠

Citas Recibidas en Web of Science: 44

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Keywords

  • Porto-sinusoidal vascular disorder; idiopathic portal hypertension; natural history

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