Premkumar Madhumita, Anand Anil C
Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Department of Hepatology, Kalinga Institute of Medical Sciences, Bhubaneshwar, Odisha, India.
J Clin Exp Hepatol. 2024 Sep-Oct;14(5):101396. doi: 10.1016/j.jceh.2024.101396. Epub 2024 Mar 12.
Non-cirrhotic portal hypertension (NCPH) is a well-recognized clinico-pathological entity, which is associated with clinical signs and symptoms, imaging, and endoscopic features of portal hypertension (PHT), in absence of cirrhosis. In patients with NCPH without known risk factors of PHT or extrahepatic portal vein thrombosis, the condition is called idiopathic non-cirrhotic portal hypertension (INCPH). There are multiple infectious, immune related causes, systemic diseases, drug and toxin exposures, haematological disorders, and metabolic risk factors that have been associated with this INCPH. However, the causal pathogenesis is still unclear. The Vascular liver disorders interest group group recently proposed porto-sinusoidal vascular disease (PSVD) as a syndromic entity, which provides definite histopathological criteria for diagnosis of NCPH (table 1). The three classical histo-morphological lesions specific for PSVD include obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. The PSVD definition includes patients with portal vein thrombosis, PVT, and even those without PHT, thus broadening the scope of diagnosis to include patients who may have presented early, prior to haemodynamic changes consistent with PHT. However, this new diagnosis has pros and cons. The cons include mandating invasive liver biopsy to assess the PSVD histological triad in all patients with NCPH, an erstwhile clinical diagnosis in Asian patients. In addition, the natural history of the subclinical forms of PSVD without PHT and linear progression to develop PHT is unknown yet. In this review, we discuss the diagnosis and treatment of INCPH/PSVD, fallacies and strengths of the old and new schema, pathobiology of this disease, and clinical correlates in an Asian context. Although formulation of standardised diagnostic criteria is useful for comparison of clinical cohorts with INCPH/PSVD, prospective clinical validation in global cohorts is necessary to avoid misclassification of vascular disorders of the liver.
非肝硬化性门静脉高压症（NCPH）是一种公认的临床病理实体，在无肝硬化的情况下，它与门静脉高压症（PHT）的临床体征和症状、影像学及内镜特征相关。在无已知PHT风险因素或肝外门静脉血栓形成的NCPH患者中，这种情况被称为特发性非肝硬化性门静脉高压症（INCPH）。有多种感染性、免疫相关病因、全身性疾病、药物和毒素暴露、血液系统疾病以及代谢风险因素与这种INCPH相关。然而，其因果发病机制仍不清楚。血管性肝病兴趣小组最近提出将门静脉窦状血管疾病（PSVD）作为一种综合征实体，它为NCPH的诊断提供了明确的组织病理学标准（表1）。PSVD特有的三种经典组织形态学病变包括闭塞性门静脉病、结节状再生性增生和不完全性间隔纤维化。PSVD的定义包括门静脉血栓形成（PVT）患者，甚至包括那些无PHT的患者，从而扩大了诊断范围，将那些可能在与PHT一致的血流动力学变化之前就已早期出现症状的患者纳入其中。然而，这种新诊断方法有其优缺点。缺点包括要求对所有NCPH患者进行侵入性肝活检以评估PSVD组织学三联征，而在亚洲患者中这曾是一种临床诊断方法。此外，无PHT的PSVD亚临床形式的自然病程以及发展为PHT的线性进展尚不清楚。在本综述中，我们将在亚洲背景下讨论INCPH/PSVD的诊断和治疗、新旧模式的谬误与优势、该疾病的病理生物学以及临床相关性。尽管制定标准化诊断标准有助于比较INCPH/PSVD临床队列，但有必要在全球队列中进行前瞻性临床验证，以避免肝脏血管疾病的错误分类。
