Surgical Pathology & Cytopathology Unit, Department of Medicine - DIMED, University of Padova, Padua, Italy.
Department of Internal Medicine, San Pietro Hospital, Ponte San Pietro, Italy.
Virchows Arch. 2018 Jul;473(1):23-31. doi: 10.1007/s00428-018-2355-8. Epub 2018 Apr 12.
Idiopathic non-cirrhotic portal hypertension is an under-recognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non-cirrhotic portal hypertension. However, the diagnosis is often difficult because the disease resembles cirrhosis and there is no gold standard test. Liver biopsy is an essential tool: it is able to exclude cirrhosis and other causes of portal hypertension and it allows the identification of the characteristic lesions. Nonetheless, the histological diagnosis of idiopathic non-cirrhotic portal hypertension is not always straightforward, in particular by needle biopsy samples, because there is no pathognomonic lesion, but rather a variety of vascular changes which are unevenly distributed, very subtle, and not all necessarily identified in a single specimen. Pathologists should be able to recognize several patterns of injury, involving portal/periportal areas as well as parenchymal structures.The histological features of idiopathic non-cirrhotic portal hypertension are described in this review, focusing on their interpretation in needle biopsy specimens.
特发性非肝硬化性门静脉高压症是一种未被充分认识的血管性肝脏疾病，其特征为在无肝硬化的情况下出现门静脉高压的临床征象。根据定义，任何已知可导致无肝硬化性门静脉高压的疾病以及任何慢性肝病的病因均需排除，以作出特发性非肝硬化性门静脉高压症的诊断。然而，由于该疾病类似于肝硬化且缺乏金标准检测，因此诊断通常较为困难。肝活检是一种重要的工具：它能够排除肝硬化和其他门静脉高压的病因，并可识别特征性病变。尽管如此，特发性非肝硬化性门静脉高压症的组织学诊断并不总是明确的，特别是通过针芯活检样本，因为没有特征性病变，而是存在多种血管改变，这些改变分布不均、非常细微，并非所有病变在单个标本中都能识别。病理学家应该能够识别几种损伤模式，包括门脉/门周区域以及实质结构。本文重点介绍了特发性非肝硬化性门静脉高压症的组织学特征，同时关注了其在针芯活检标本中的解读。
