特发性门静脉高压症的诊治现状

特发性门静脉高压症(IPH)非常罕见,其特点是有门静脉高压,但没有肝硬化,有门静脉小分支堵塞或狭窄,却没有肝静脉和门静脉主干的堵塞。该疾病尚有其它命名,如:特发性非硬化性门静脉高压症、非肝硬化性门静脉纤维化等。IPH病因及发病机制未明确,可能与遗传、免疫、感染等因素相关。临床表现上IPH以脾功能亢进、胃食管静脉曲张为主,而少有肝功能不全、肝性脑病。诊疗上尚有不少争议,目前IPH的诊断为排除性诊断,治疗上多参考肝硬化性门静脉高压的指南。IPH预后资料不多,目前认为较肝硬化性门静脉高压者预后更佳,只有少数患者进展至肝衰竭。

Current status regarding diagnosis and management of idiopathic portal hypertension

Idiopathic portal hypertension (IPH) is a very rare disease, characterized by portal hypertension with obstruction or stenosis of the intrahepatic peripheral portal branches, in the absence of liver cirrhosis or obstruction of hepatic vein and main portal vein. This condition also has other denominations such as idiopathic non-cirrhotic portal hypertension and non-cirrhotic portal fibrosis. The etiology and pathogenesis of IPH remain unclear, and may be related to heredity, immunity, infection and other factors. The main manifestations of this disease are hypersplenism and gastroesophageal varices, with less occurrence of hepatic insufficiency and hepatic encephalopathy. There are still many controversies in the diagnosis and treatment of IPH. Its diagnosis is only made by exclusion, and treatment chiefly refers to the guidelines for cirrhotic portal hypertension. There are few data on prognosis of IPH, and IPH is considered to have a better prognosis than that of cirrhotic portal hypertension, and in only a few patients may progress to liver failure.