| 摘要: |
| ［摘要］ 目的 了解原发性胆汁性肝硬化(PBC)患者的临床、免疫学和病理学特点。方法 回顾性分析44例PBC患者的一般资料、临床特点、生化检查、免疫学检查、自身抗体和病理的结果。结果 44例PBC患者男女比例1∶7.8，发病年龄为（57.68±10.71）岁，首次发病诊断分别为肝功能异常(23例，52.27%)、肝硬化(11例，25.00%)、自身免疫性肝炎(2例，4.55%)等；临床表现主要为乏力(25例，56.82%)，皮肤瘙痒(17例，38.64%)，黄疸(16例，36.36%)，口干、眼干(15例，34.09%)等。PBC重叠自身免疫性肝炎(AIH)7例（15.91%），合并其他自身免疫性疾病25例（56.82%），生化检查显示碱性磷酸酶（ALP）、γ-谷氨酰转肽（GGT）水平升高，IgM升高明显，自身抗体中以ANA、AMA、AMA-M2阳性者为主。肝活检25例，17例可见肝小叶内散在点、片状坏死，20例汇管区碎屑状坏死，21例汇管区淋巴细胞浸润，11例细小胆管增生，16例纤维组织增生，6例假小叶形成，6例胆管上皮变性，7例胆管炎性细胞浸润，3例肝小叶内及汇管区见肉芽肿形成，5例小叶间胆管消失，3例羽毛状变性，2例胆栓形成，6例小叶间胆管炎。结论 PBC女性多见，发病和临床表现无特异性，肝功能检查以肝汁淤积为主，AMA、AMA-M2阳性可诊断。肝组织学改变小叶间胆管炎、胆管数目减少,汇管区淋巴细胞聚集、肉芽肿形成、细小胆管增生,以及肝细胞羽毛状变性是主要病理特点。 |
| 关键词: 原发性胆汁性肝硬化 免疫学 病理学 临床特征 |
| DOI：10.3969/j.issn.1674-3806.2016.10.09 |
| 分类号:R 657.3 |
| 基金项目: |
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| Clinical, immunological and pathological characteristics of primary biliary cirrhosis: a repert of 44 cases |
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WANG Hai-xia, ZHANG Cai-cai, SONG Qi-shuang
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Qingdao University of Science and Technology, Shangdong 266061, China
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| Abstract: |
| ［Abstract］ Objective To summarize the clinical, immunological and pathological features of primary biliary cirrhosis(PBC).Methods The data of 44 PBC patients were retrospectively analyzed, including the general information, clinical characteristics, and results of the biochemical, immunological and pathological examinations.Results Most of the PBC patients were women,with a mean age of (57.68±10.71)years, and the ratio of male to female was 1∶7.8. Of all the patients, 52.27% were presented with abnormal liver function hepatitis, 25.00% with cirrhosis and 11.36% with autoimmune liver diseases. The main clinical manifestations included fatigue(56.82%), pruritus(38.64%), jaundice(84.5%), xerostomia and keratoconjunctivitis sicca(34.09%). PBC complicated with autoimmune hepatitis(AIH) covered in 15.91% patients, and those complicated with other autoimmune diseases accounted for 56.82%. Biochemical examination showed the elevated levels of serum alkaline phosphatase(ALP) and serum gamma glutamyl transpeptidase(GGT) in PBC patients. High levels of immunoglobulin M(IgM) were tested in most PBC patients. The positive percentage of antinuclear antibody(ANA) was 95.45%, and antimitochondrial antibody(AMA) and antibody of anti-mitochondrial M2 subtype(AMA-M2) were 90. 91%. In 25 patients who received liver biopsy, the main histopathological changes were: punctiform and focus-form necrosis, piecemeal necrosis, necroinflammation of interbobular bile ducts, smaller bile duct proliferation, fibrosis, pseudolobular formation inflammation aggregation around small bile duct, anuloma formation, small bile duct vanish, feathery degeneration of hepatocytes, bilirubi-nostasis in canaliculi and cholangitis of interlobular bile ducts.Conclusion PBC occurs more frequently in females than in males, and the onset and clinical features are not typical. The liver function test reveals acholestatic pattern. Positive AMA/ AMA-M2 may be crucial to the diagnosis of PBC.The histopathologial hallmorks of PBC are mecroinflammation and ductopenia involved mainly in the interlobular bile ducts, lymphocyte aggregation,granuloma formation and bile ductular proliferation in the portal area; and feathery degeneration of the hepatocytes. |
| Key words: Primary biliary cirrhosis Immunology Pathology Clinical characteristic |
