Idiopathic portal hypertension: Natural history and long-term outcome

作者：Sith Siramolpiwat, Susana Seijó, Rosa Miquel, Annalisa Berzigotti, Ángeles García‐Criado, Anna Darnell, Fanny Turón, Virginia Hernández–Gea, Jaume Bosch, Juan Carlos García‐Pagán · 发表于：Hepatology · 年份：2013 · DOI：10.1002/hep.26904 · 被引用次数：204 · 研究领域：Liver Disease and Transplantation、Organ Transplantation Techniques and Outcomes、Hepatitis Viruses Studies and Epidemiology

UNLABELLED: Idiopathic portal hypertension (IPH) is a rare cause of intrahepatic portal hypertension. Data on natural history and prognosis of IPH are limited. We sought to describe the complications and long-tem outcome of IPH by retrospectively studying 69 biopsy-proven cases of IPH. Mean duration of follow-up was 6.7 ± 4.6 years. All patients had evidence of portal hypertension (PH) at diagnosis, and 42% were symptomatic. Variceal bleeding (VB) was the most common manifestation. In those without bleeding at diagnosis, 74% had varices at first endoscopy. In those with large varices, the 1-year probability of first bleeding despite primary prophylaxis was 9%. The 1-year probability of rebleeding was 22%. Ascites and hepatic encephalopathy was documented in 26% and 7% of patients, respectively, at least once during the clinical course. The 1-year probability of developing portal vein thrombosis (PVT) was 9%, and 53% of patients receiving anticoagulation achieved recanalization. Human immunodeficiency virus (HIV) infection and VB at diagnosis were the independent predictors of PVT. Seven patients died (6 as a result of an IPH-related cause) and 2 were transplanted. Probability of liver transplantation-free survival was 82% at 10 years. Presence of a severe associated disorder and ascites as a presenting symptom were associated with poor survival. CONCLUSION: Variceal bleeding is a major complication of IPH. Using, in IPH patients, the same management approach for PH as in cirr...