Mountain Vista Medical Center, Midwestern University Program, Mesa AZ, USA.
Penn State College of Medicine, Hershey, PA, USA.
Sci Prog. 2024 Jul-Sep;107(3):368504241264996. doi: 10.1177/00368504241264996.
Portal hypertension (PH) is a clinically significant entity that could present with life-threatening gastrointestinal bleeding. Cirrhosis is the most common cause of PH, with well-documented histopathology and etiology. However, in idiopathic portal hypertension (IPH), no single histopathologic finding is associated with PH. Our systematic review aims to identify and summarize the prevalence of the common histological findings of IPH.
We systematically searched PubMed, Cochrane CENTRAL, Web of Science, and Scopus till 1ST March 2022 for studies describing the histopathological features of IPH. Data were extracted from eligible studies and pooled as events rate and 95% confidence interval (CI) using binary random-effects model by open meta-analyst software.
We included 23 retrospective studies with a total sample size of 813 patients. The overall incidence of nodular regenerative hyperplasia was 38.6%, 59.8% for portal fibrosis, 51.3% for periportal fibrosis, 39.3% for perisinusoidal fibrosis, 89.8% for portal vein sclerosis, 42.2% for portal inflammation, 53.3% for mega-sinusoids, 39.5% for thickening of portal vein branches, 93.8% for narrowing of portal veins, 53.3% for hepatic veins/venous outflow obstruction, 51.4% for aberrant portal/periportal vessels, 42.4% for shunt vessel, 50.9% for ductular proliferation, and 16.3% for steatosis.
Due to the relatively non-pathognomonic and non-specific nature of IPH, a combination of different histological features such as the portal and periportal fibrosis, portal vein sclerosis, mega-sinusoids, narrowing of portal veins, hepatic venous outflow obstruction, aberrant portal or periportal vessels, and ductular proliferation may be of value in diagnosing IPH as the incidence rate of these features was at approximately 50%.
门静脉高压（PH）是一种具有临床意义的病症，可能导致危及生命的胃肠道出血。肝硬化是 PH 的最常见原因，其具有明确的组织病理学和病因。然而，在特发性门静脉高压（IPH）中，没有任何单一的组织病理学发现与 PH 相关。我们的系统评价旨在确定和总结 IPH 的常见组织学表现的患病率。
我们系统地检索了 PubMed、Cochrane 中心、Web of Science 和 Scopus，截至 2022 年 3 月 1 日，以查找描述 IPH 组织病理学特征的研究。从合格的研究中提取数据，并使用开放元分析软件的二项随机效应模型汇总为事件发生率和 95%置信区间（CI）。
我们纳入了 23 项回顾性研究，共有 813 名患者的总样本量。结节性再生性增生的总体发生率为 38.6%，门静脉纤维化为 59.8%，门周纤维化为 51.3%，窦周纤维化为 39.3%，门静脉硬化为 89.8%，门静脉炎症为 42.2%，巨窦为 53.3%，门静脉分支增厚为 39.5%，门静脉狭窄为 93.8%，肝静脉/静脉流出道阻塞为 53.3%，异常门/门周血管为 51.4%，分流血管为 42.4%，胆管增生为 50.9%，脂肪变性为 16.3%。
由于 IPH 的相对非特征性和非特异性性质，结合不同的组织学特征，如门周和门周纤维化、门静脉硬化、巨窦、门静脉狭窄、肝静脉流出道阻塞、异常门或门周血管和胆管增生，可能有助于诊断 IPH，因为这些特征的发生率约为 50%。
