Bihari Chhagan, Dhariwal Sneha, Shasthry Saggere Murlikrishna, Rastogi Archana, Sharma Manoj Kumar, Sarin Shiv Kumar
Institute of Liver and Biliary Sciences, New Delhi, Delhi, India
Institute of Liver and Biliary Sciences, New Delhi, Delhi, India.
J Clin Pathol. 2025 Feb 18;78(3):169-176. doi: 10.1136/jcp-2023-209321.
Portosinusoidal vascular disease (PSVD) is a broad term encompassing varied histological patterns with changes in portal tracts and sinusoids without cirrhosis. We aimed to assess whether there is any clinical and pathological difference among the various histological categories of PSVD.
This study included liver biopsy cases classified as PSVD (2020-2022). Clinical and laboratory parameters were obtained from the electronic records. PSVD cases were histologically categorised as obliterative portal venopathy (OPV), OPV with fibrosis (OPV-F), incomplete septal cirrhosis (ISC), nodular regenerative hyperplasia (NRH), mega sinusoids with fibrosis (MSF) and unclassified. Follow-up complications were recorded.
PSVD categories were OPV (45 (26%)), OPV-F (37 (21.4%)), ISC (20 (11.6%)), NRH (19 (11%)), MSF (19 (11%)) and unclassified (33 (19%)). Elevated hepatic venous pressure gradient (HVPG) was noted in OPV-F (10 (IQR: 12-14.7)) and ISC (12 (IQR: 9-14)) mm Hg with higher fibrosis quantity in liver tissue and elevated procollagen III aminoterminal propeptide, which correlated with HVPG. On immunohistochemistry, OPV-F and ISC showed lesser expression of ADAMT13 in liver biopsies (p<0.001). On follow-up, ascites development was more in OPV-F and ISC than in other categories (p=0.001). Higher liver stiffness measurement (LSM) values were recorded in MSF and NRH, compared with other categories, but it did not correlate with fibrosis in liver biopsy.
OPV-F and ISC had higher HVPG, fibrosis, and more ascites development on follow-up than the other categories of PSVD, and all are not the same. In contrast, MSF and NRH have spuriously high LSM.
门静脉窦状隙血管疾病(PSVD)是一个广义术语,涵盖门静脉区域和肝血窦发生变化但无肝硬化的多种组织学模式。我们旨在评估PSVD的各种组织学类型之间是否存在临床和病理差异。
本研究纳入了2020年至2022年期间分类为PSVD的肝活检病例。从电子记录中获取临床和实验室参数。PSVD病例在组织学上分为闭塞性门静脉病(OPV)、伴有纤维化的OPV(OPV-F)、不完全性间隔性肝硬化(ISC)、结节性再生性增生(NRH)、伴有纤维化的大肝血窦(MSF)以及未分类。记录随访并发症。
PSVD的类型包括OPV(45例(26%))、OPV-F(37例(21.4%))、ISC(20例(11.6%))、NRH(19例(11%))、MSF(19例(11%))和未分类(33例(19%))。在OPV-F(10(四分位间距:12 - 14.7))和ISC(12(四分位间距:9 - 14))毫米汞柱中观察到肝静脉压力梯度(HVPG)升高,肝组织纤维化量增加且前胶原III氨基端前肽升高,这与HVPG相关。免疫组化显示,OPV-F和ISC在肝活检中ADAMT13表达较低(p<0.001)。随访时,OPV-F和ISC的腹水发生率高于其他类型(p = 0.001)。与其他类型相比,MSF和NRH的肝脏硬度测量(LSM)值更高,但与肝活检中的纤维化无关。
与其他PSVD类型相比,OPV-F和ISC在随访时具有更高的HVPG、纤维化以及更多的腹水发生,且各不相同。相比之下,MSF和NRH的LSM值异常高。