Fiordaliso Michele, Marincola Giuseppe, Pala Barbara, Muraro Raffaella, Mazzone Mariangela, Di Marcantonio Maria Carmela, Mincione Gabriella
Department of Medicine and Ageing Sciences, University "G. D'Annunzio" of Chieti-Pescara, Via dei Vestini 29, 66100 Chieti, Italy.
Bariatric and Metabolic Surgery Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo Agostino Gemelli 8, 00168 Rome, Italy.
Diagnostics (Basel). 2023 Oct 20;13(20):3263. doi: 10.3390/diagnostics13203263.
Non-cirrhotic portal hypertension (NCPH), also known as idiopathic non-cirrhotic portal hypertension (INCPH) and porto-sinusoidal vascular disorder (PSVD), is a rare disease characterized by intrahepatic portal hypertension (IPH) in the absence of cirrhosis. The precise etiopathogenesis of IPH is an area of ongoing research. NCPH diagnosis is challenging, as there are no specific tests available to confirm the disease, and a high-quality liver biopsy, detailed clinical information, and an expert pathologist are necessary for diagnosis. Currently, the treatment of NCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied that aimed to modify the natural history of the disease; however, transjugular intrahepatic porto-systemic shunt (TIPS) placement, shunt and liver transplantation are considerable symptomatic options. In this review, we discuss the heterogeneity of NCPH as well as its etiopathogenesis, clinical presentation and management issues. Starting from the assumption that portal hypertension does not always mean cirrhosis, cooperative studies are probably needed to clarify the issues of etiology and the possible genetic background of this rare disease. This knowledge might lead to better treatment and perhaps better prevention.
非肝硬化性门静脉高压症（NCPH），也被称为特发性非肝硬化性门静脉高压症（INCPH）和门静脉-肝窦血管疾病（PSVD），是一种罕见疾病，其特征为在无肝硬化的情况下出现肝内门静脉高压（IPH）。IPH的确切病因发病机制是一个正在研究的领域。NCPH的诊断具有挑战性，因为没有可用于确诊该疾病的特异性检查，高质量的肝活检、详细的临床信息以及专业病理学家对于诊断都是必要的。目前，NCPH的治疗依赖于按照肝硬化门静脉高压的现行指南预防与门静脉高压相关的并发症。尚未有旨在改变该疾病自然病程的治疗方法得到研究；然而，经颈静脉肝内门体分流术（TIPS）置入、分流术和肝移植是重要的对症治疗选择。在本综述中，我们讨论了NCPH的异质性及其病因发病机制、临床表现和管理问题。从门静脉高压并不总是意味着肝硬化这一假设出发，可能需要开展合作研究以阐明该罕见疾病的病因问题以及可能的遗传背景。这些知识可能会带来更好的治疗方法，或许还能实现更好的预防。
