肝脏 ›› 2018, Vol. 23 ›› Issue (9): 769-771.

• 论著 • 上一篇    下一篇

自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征患者肝组织病理学特征

尧颖, 高建鹏, 王辉, 张志波, 翟慧勤, 徐智媛   

  1. 650051 昆明医科大学附属延安医院消化科(尧颖,高建鹏,王辉,张志波,翟慧勤);昆明医科大学第二附属医院消化科(徐智媛)
  • 收稿日期:2018-03-19 出版日期:2018-09-30 发布日期:2020-04-26
  • 通讯作者: 徐智媛,Email: xuzhiyuan0109@163.com
  • 基金资助:
    云南省昆明市卫计委卫生科技项目资助(2017-03-03-001)

Pathological characteristics of the overlap syndrome of autoimmune hepatitis and primary biliary cholangitis

YAO Ying, GAO Jian-peng, WANG Hui, ZHANG Zhi-bo, ZHAI Hui-qin, XU Zhi-yuan   

  1. Department of Gastroenterology, Yanan Hospital Affiliated Kunming Medical University, Kunming, Yunnan, China; Department of Gastroenterology, The Second Affiliated Hospital, Kunming Medical University, Kunming, Yunnan,China
  • Received:2018-03-19 Online:2018-09-30 Published:2020-04-26
  • Contact: XU Zhi-yuan, Email: xuzhiyuan0109@163.com

摘要: 目的 分析自身免疫性肝炎-原发性胆汁性胆管炎(AIH-PBC)重叠综合征的组织病理学变化,提高对AIH-PBC重叠综合征的临床及病理学特征的认识。 方法 对10例AIH-PBC重叠综合征患者的肝穿刺组织病理资料进行回顾性分析。 结果 AIH-PBC重叠综合征患者多发于中年女性(80%),肝组织病理变化兼具界面性肝炎,桥接样坏死及小胆管减少甚至消失。 结论 自身免疫性肝病在中国人中日趋增多,AIH-PBC重叠综合征并非罕见,其诊断需综合临床表现、生化、免疫指标和组织学变化,重叠综合征兼具AIH和PBC的组织病理学特征,均可见肝小叶界面炎,且多为中重度,可见桥接样坏死及小叶中央融合性坏死,肝细胞炎症活动度从中度到重度不等,纤维化程度从S2到S4,同时均有不同程度的小胆管病变,其中3例有明显胆管数目减少,2例有细小胆管增生。

关键词: 自身免疫性肝炎, 原发性胆汁性胆管炎, 重叠综合征, 病理学

Abstract: Objective To analyze the histopathological changes of autoimmune hepatitis (AIH)-primary biliary cholangitis (PBC) overlap syndrome and to improve the understanding of its clinical and pathological features.Methods The pathological changes of liver tissue in 10 patients with AIH-PBC overlap syndrome were retrospectively analyzed. Results The AIH-PBC overlap syndrome was more common in middle-aged women (80%) presenting with clinical and pathological characteristics of both AIH and PBC. Interface hepatitis, bridging necrosis and central confluence necrosis were all observed. The inflammatory activity of the liver tissue was from moderate to severe and fibrosis score was from S2 to S4. At the same time, different degrees of small bile duct lesions were observed, including 3 cases with obvious bile ducts decrease and 2 cases with small bile duct hyperplasia.Conclusion Diagnosis of AIH-PBC overlap syndrome should be based on the clinical presentation and changes in biochemistry, immunology and histology. The overlap syndrome has the histopathological features of both AIH and PBC, with main histological changes of interface hepatitis, bridging necrosis and small bile duct reduction.

Key words: Autoimmune hepatitis, Primary biliary cholangitis, Overlap syndrome, Pathology