Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Texas Medical Branch, Galveston, TX, USA.
Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Texas Medical Branch, Galveston, TX, USA.
Respir Med. 2022 Oct;202:106969. doi: 10.1016/j.rmed.2022.106969. Epub 2022 Aug 29.
Portopulmonary hypertension (POPH) is a complication of cirrhosis that results in right ventricular failure and death. The objective of this autopsy investigation was to compare pulmonary arterial receptors in subjects with cirrhosis and histopathologic evidence of pulmonary vascular disease (PVD) and control group subjects with cirrhosis lacking evidence of PVD. Autopsy records of 824 subjects with cirrhosis were reviewed to identify pulmonary arterial vasculopathy. Lung sections from paraffin embedded blocks were immunostained for endothelin A (ET-A), endothelin B (ET- B), estrogen α (ER-α), estrogen β (ER-β), and vascular endothelial growth factor (VEGF). Subjects with cirrhosis and histopathologic evidence of PVD included 27 individuals with intimal hyperplasia (93%), medial hypertrophy (96%), and plexiform lesions (78%). Immunohistochemical staining for ET-A revealed positive reactivity in 40% of the group with cirrhosis and histopathologic evidence of PVD and 13% of the control group (NS). ET-B reactivity in the pulmonary endothelium and smooth muscle was identified in all subjects with cirrhosis and histopathologic evidence of PVD and control group. VEGF reactivity was identified in the endothelium in all subjects with cirrhosis and histopathologic evidence of PVD compared with 33% of the control group (p = 0.0002). ER-β reactivity was observed in four subjects (26.6%) with cirrhosis and histopathologic evidence of PVD while none in the control group (NS). Cirrhosis and histopathologic evidence of PVD was found in 3.3% of autopsies with the pulmonary vasculature immunohistochemical profile demonstrating endothelial and smooth muscle reactivity for endothelin, VEGF and ER-β.
肝肺高血压（POPH）是肝硬化的一种并发症，可导致右心室衰竭和死亡。本尸检研究的目的是比较肝硬化伴有和不伴有肺血管疾病（PVD）组织病理学证据的患者的肺动 脉受体。对 824 例肝硬化患者的尸检记录进行了回顾，以确定肺动脉血管病变。对石蜡包埋块的肺组织切片进行内皮素 A（ET-A）、内皮素 B（ET-B）、雌激素 α（ER-α）、雌激素 β（ER-β）和血管内皮生长因子（VEGF）的免疫染色。伴有 PVD 组织病理学证据的肝硬化患者包括 27 例内膜增生（93%）、中膜肥厚（96%）和丛状病变（78%）患者。ET-A 的免疫组织化学染色显示，有组织病理学证据的 PVD 组中 40%的患者和对照组中 13%的患者（无统计学意义）有阳性反应。所有伴有 PVD 组织病理学证据的肝硬化患者和对照组的肺内皮和平滑肌中均发现 ET-B 反应。所有伴有 PVD 组织病理学证据的肝硬化患者的内皮中均发现 VEGF 反应，而对照组中只有 33%的患者有反应（p=0.0002）。在伴有 PVD 组织病理学证据的 4 例肝硬化患者（26.6%）中观察到 ER-β 反应，而对照组中无一例患者有反应（无统计学意义）。在 3.3%的尸检中发现了肝硬化和 PVD 的组织病理学证据，其肺血管免疫组织化学特征显示内皮和平滑肌对内皮素、VEGF 和 ER-β 有反应。
