Department of Pathology, Medical School, Democritus University of Thrace, Alexandroupolis, Greece.
Eur J Gastroenterol Hepatol. 2021 Dec 1;33(12):e7-e12. doi: 10.1097/MEG.0000000000001876.
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an autoimmune, slowly progressive, cholestatic liver disease characterized by nonsuppurative destructive cholangitis, and interlobular bile duct destruction. Necroinflammatory activities of the hepatic parenchyma and limiting plates of milder form along with late liver fibrosis may develop. Serum liver tests include elevated serum alkaline phosphatase along with a positive antimitochondrial antibody (AMA) in nearly 95% of patients. Liver biopsies are an important confirmatory and staging tool and are additionally very helpful when AMA is negative. More specifically, the earliest changes in liver biopsy suspicious for PBC can be detected, namely loss of the canals of Hering (CoH), as proposed by various authors recently. CoH loss has been described as an early feature of PBC. We focus on early histologic features of PBC, investigating through the literature the possible role of 'minimal change' supporting the clinical diagnosis of PBC, even in the absence of characteristic granulomatous duct destructive lesions.
原发性胆汁性胆管炎（PBC），以前称为原发性胆汁性肝硬化，是一种自身免疫性、缓慢进展性、胆汁淤积性肝病，其特征为非化脓性破坏性胆管炎和小叶间胆管破坏。肝实质和较轻微的门管区限局性板层炎症坏死活动以及晚期肝纤维化可能会发展。血清肝脏检查包括血清碱性磷酸酶升高，近 95%的患者抗线粒体抗体（AMA）阳性。肝活检是一种重要的确认和分期工具，当 AMA 阴性时也非常有帮助。更具体地说，可以检测到肝活检中疑似 PBC 的最早变化，即 Hering 管（CoH）丢失，这是最近由多位作者提出的。CoH 丢失已被描述为 PBC 的早期特征。我们关注 PBC 的早期组织学特征，通过文献研究探讨“微小变化”在支持 PBC 的临床诊断中的可能作用，即使在没有特征性的肉芽肿性胆管破坏病变的情况下也是如此。
