Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan.
Hepatol Res. 2009 Oct;39(10):1023-31. doi: 10.1111/j.1872-034X.2009.00555.x.
Idiopathic portal hypertension (IPH) is a non-cirrhotic presinuosidal portal hypertension of unknown etiology. Stenosis of smaller portal veins with portal fibrosis is a pathologic hallmark of IPH. Association of systemic sclerosis (SSc) with IPH is recognized, and similar pathologic features are reported in small portal tracts and skin of IPH and SSc, respectively. In addition, levels of transforming growth factor-beta (TGF-beta) and connective tissue growth factor are elevated in serum and in affected skin and portal tracts of these two diseases, suggesting that IPH share fibrogenetic mechanisms with SSc. Endothelial to mesenchymal transition (EndMT) of microvasculatures of skin could be responsible for dermal fibrosis in SSc. In IPH, EndMT of portal vein endothelium via TGF-beta/Smad activation may also be involved in small portal venpathy. In IPH, enhanced expression of pSmad2 in venous endothelium of smaller portal veins was associated with reduced CD34 expression. CD34 and S100A4, and CD34 and type I collagen were colocalized to portal vein endothelium in IPH. Such myofibroblastic phenotypes may be responsible for periportal-venous deposition of collagen and compressive portal venous obliteration. These small portal venous lesions may in turn lead to portal venous insufficiency followed by subcapsular atrophy in IPH.
特发性门静脉高压症（IPH）是一种不明病因的非肝硬化性前窦门静脉高压症。较小门静脉的狭窄伴门静脉纤维化是 IPH 的病理特征。系统性硬化症（SSc）与 IPH 相关，在 IPH 和 SSc 的小门静脉和皮肤中分别报道了类似的病理特征。此外，转化生长因子-β（TGF-β）和结缔组织生长因子在血清中和这两种疾病的受累皮肤和门静脉中的水平升高，表明 IPH 与 SSc 具有纤维生成机制。皮肤微血管内皮到间充质转化（EndMT）可能是 SSc 皮肤纤维化的原因。在 IPH 中，通过 TGF-β/Smad 激活的门静脉内皮的 EndMT 也可能参与小门静脉病变。在 IPH 中，较小门静脉静脉内皮中 pSmad2 的表达增强与 CD34 表达减少相关。在 IPH 中，CD34 和 S100A4，以及 CD34 和 I 型胶原均与门静脉内皮共定位。这种成纤维细胞表型可能是导致胶原在门脉周围静脉沉积和压迫性门静脉闭塞的原因。这些小的门静脉病变反过来可能导致 IPH 中的门静脉功能不全，随后出现包膜下萎缩。
