Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.
Department of Pathology, San Camillo Forlanini Hospital, Rome, Italy.
Dig Liver Dis. 2024 Feb;56(2):291-296. doi: 10.1016/j.dld.2023.07.023. Epub 2023 Aug 5.
The term porto-sinusoidal vascular disorder (PSVD) was recently proposed to replace that of idiopathic non-cirrhotic portal hypertension (INCPH) to describe patients with typical histological lesions in absence of cirrhosis, irrespective of the presence/absence of portal hypertension (PH), and new diagnostic criteria were defined. The study aimed to compare the applicability between the diagnostic criteria of PSVD and those of INCPH.
53 patients affected by PSVD were enrolled. Biochemical, clinical, ultrasound and histological data, the presence and type of associated diseases were recorded in a database. According to the new criteria, histological data and signs of PH were divided into specific and non-specific. Percutaneous and transjugular biopsies were compared to establish the usability of the two methods for diagnostic purposes.
In 85% of the patients the diagnosis of PSVD was obtained by applying the first criterion (25 had specific histological signs with specific signs of PH); one patient presented with specific histological signs but no PH. In 8 patients the diagnosis was obtained by applying the second criterion. 19% of patients had portal vein thrombosis. Finally, the prevalence of the various histological lesions was similar between the patients submitted to percutaneous and transjugular liver biopsy.
The study confirms that the diagnostic criteria of PSVD lead to the inclusion of a greater number of patients than INCPH.
最近提出了门-体静脉血管障碍(PSVD)这一术语,以取代特发性非肝硬化性门静脉高压症(INCPH),用于描述存在典型组织学病变而无肝硬化的患者,无论是否存在门静脉高压症(PH),并定义了新的诊断标准。本研究旨在比较 PSVD 和 INCPH 诊断标准的适用性。
纳入了 53 例 PSVD 患者。在数据库中记录了生化、临床、超声和组织学数据、并存疾病的存在和类型。根据新标准,将组织学数据和 PH 征象分为特异性和非特异性。比较经皮和经颈静脉肝活检以确定两种方法在诊断目的上的可用性。
在 85%的患者中,通过应用第一个标准(25 例具有特定组织学征象和特定 PH 征象)诊断为 PSVD;1 例患者具有特定组织学征象但无 PH。在 8 例患者中,应用第二个标准诊断为 PSVD。19%的患者存在门静脉血栓形成。最后,接受经皮和经颈静脉肝活检的患者的各种组织学病变的患病率相似。
本研究证实,PSVD 的诊断标准导致纳入的患者数量多于 INCPH。