Okudaira Masahiko, Ohbu Makoto, Okuda Kunio
Department of Pathology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.
Semin Liver Dis. 2002 Feb;22(1):59-72. doi: 10.1055/s-2002-23207.
Idiopathic portal hypertension (IPH) is a disorder of unknown etiology, clinically characterized by portal hypertension (varices and portosystemic collateral vessels), splenomegaly, and anemia (hypersplenism). A similar disorder is called noncirrhotic portal fibrosis in India, and hepatoportal sclerosis seems to be the counterpart in the United States. This disease is uncommon in developed countries. Middle-aged women are more prone to IPH in Japan. The liver has no cirrhosis or pseudonodule formation, and the principal pathologic changes are considerable portal fibrosis, devastation of intrahepatic terminal portal radicles, and parenchymal atrophy of the liver secondary to portal malperfusion. The characteristic portal hemodynamics include intrahepatic presinusoidal portal hypertension, increased splenic and portal vein blood flow, and increased intrahepatic portal resistance. The prognosis is generally good depending on the management of bleeding varices. Although the etiology is obscure, certain immunologic abnormalities seem to play an etiologic role in Japanese patients, and the incidence has markedly declined in recent years in Japan, indirectly suggesting a role of infection. The theory that IPH represents an undiagnosed intrahepatic portal vein thrombosis is refuted.
特发性门静脉高压症（IPH）是一种病因不明的疾病，临床特征为门静脉高压（静脉曲张和门体侧支血管）、脾肿大和贫血（脾功能亢进）。在印度，类似的疾病被称为非肝硬化性门静脉纤维化，在美国则似乎对应肝门静脉硬化。这种疾病在发达国家并不常见。在日本，中年女性更容易患IPH。肝脏无肝硬化或假结节形成，主要病理变化为显著的门静脉纤维化、肝内终末门静脉分支破坏以及继发于门静脉灌注不良的肝实质萎缩。其特征性的门静脉血流动力学包括肝内窦前性门静脉高压、脾静脉和门静脉血流增加以及肝内门静脉阻力增加。根据曲张静脉出血的处理情况，总体预后良好。尽管病因不明，但某些免疫异常似乎在日本患者中起病因作用，近年来日本的发病率明显下降，间接提示感染的作用。IPH代表未被诊断的肝内门静脉血栓形成这一理论已被驳斥。
