Porto-sinusoidal vascular liver disorder with portal hypertension: Natural history and long-term outcome.
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Magaz Marta, Giudicelli-Lett Heloïse, Abraldes Juan G, Nicoară-Farcău Oana, Turon Fanny, Rajoriya Neil, Goel Ashish, Raymenants Karlien, Hillaire Sophie, Téllez Luis, Elkrief Laure, Procopet Bogdan, Orts Lara, Nery Filipe, Shukla Akash, Larrue Hélène, Degroote Helena, Aguilera Victoria, Llop Elba, Turco Laura, Indulti Federica, Gioia Stefania, Tosetti Giulia, Bitto Niccolò, Becchetti Chiara, Alvarado Edilmar, Roig Cristina, Diaz Raquel, Praktiknjo Michael, Konicek Anna-Lena, Olivas Pol, Fortea José Ignacio, Masnou Helena, Puente Ángela, Ardèvol Alba, Navascués Carmen A, Romero-Gutiérrez Marta, Scheiner Bernhard, Semmler Georg, Mandorfer Mattias, Damião Filipe, Baiges Anna, Ojeda Asunción, Simón-Talero Macarena, González-Alayón Carlos, Díaz Alba, García-Criado Ángeles, De Gottardi Andrea, Hernández-Guerra Manuel, Genescà Joan, Drilhon Nicolas, Noronha Ferreira Carlos, Reiberger Thomas, Rodríguez Manuel, Morillas Rosa María, Crespo Javier, Trebicka Jonel, Bañares Rafael, Villanueva Càndid, Berzigotti Annalisa, Primignani Massimo, La Mura Vincenzo, Riggio Oliviero, Schepis Filippo, Verhelst Xavier, Calleja José Luis, Bureau Christophe, Albillos Agustín, Nevens Frederik, Hernández-Gea Virginia, Tripathi Dhiraj, Rautou Pierre-Emmanuel, García-Pagán Juan Carlos, ERN RARE-LIVER; a study of VALDIG, an EASL consortium
Аннотация
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.
IMPACT AND IMPLICATIONS: Porto-sinusoidal vascular liver disorder (PSVD) is a rare entity that usually affects young people, frequently causes severe complications of portal hypertension, and may reduce life expectancy. To date, there is scarce information regarding its clinical manifestations, natural history and prognostic factors. The present study, including the largest number of patients with PSVD reported so far, shows that overall, when managed at centers of expertise, the prognosis of patients with PSVD is good, with LT-free survival rates of 83% and 72% at 5 and 10 years, respectively. Presence and severity of an underlying associated condition, presence of ascites, age and bilirubin, albumin and creatinine levels were associated with poor prognosis. These results are important to know for hepatologists. A final model combining these parameters enabled development of a nomogram that predicts prognosis with good discrimination and calibration capacity and can be easily applied in clinical practice.