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目的分析自身免疫性肝病临床、病理特点,探讨早期诊断、治疗方法。方法1996—1998年收治62例自身免疫性肝病,比较原发性胆汁性肝硬化(PBC)、自身免疫性肝炎(AIH)的临床症状、体征、生物化学指标及病理特征。结果自身免疫性肝病中PBC占59.68%, AIH 40.32%。自身免疫性肝病中83.8%是中老年女性,男女之比1∶10,而AIH 1∶5,发病年龄平均35岁。PBC中血循环自身免疫抗体阳性率85.29%,AIH 78.95%。在阳性病例中 AMA, AMA-M2阳性率 100%, ANA阳性率 80%,血清免疫球蛋白异常, PBC IgM增高占80%, AIHIgG增高占82.25%, P值<0.05和P值< 0.025。 PBC肝脏活组织病理显示,胆管炎和胆管增生、炎症、损伤或三者共存的特征。AIH显示门静脉区炎症、淋巴细胞、单核细胞、浆细胞浸润、坏死、纤维化。结论PBC和AIH是自身免疫性肝病临床上两个常见类型,其中AIH以Ⅰ型为主;而血中AMA-M2是PBC特征抗体,强的松龙治疗效果AIH优于PBC。
Objective To analyze the clinical and pathological features of autoimmune liver disease and to explore the methods of early diagnosis and treatment. Methods From 1996 to 1998, 62 cases of autoimmune liver disease were treated. The clinical symptoms, signs, biochemical indexes and pathological features of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) were compared. Results PBC accounted for 59.68% and AIH 40.32% in autoimmune liver disease. 83.8% of autoimmune liver disease is middle-aged and elderly women, the ratio of male to female 1:10, and AIH 1: 5, the average age of onset of 35 years old. The positive rate of circulating autoimmune antibodies in PBC was 85.29% and AIH was 78.95%. In the positive cases, AMA, AMA-M2 positive rate of 100%, ANA-positive rate of 80%, serum immunoglobulin abnormalities, PBC IgM increased 80%, AIHIgG increased 82.25%, P value <0.05 and P value <0.025. Pathology of PBC liver biopsy shows characteristics of cholangitis and biliary hyperplasia, inflammation, injury or coexistence of the three. AIH shows portal vein inflammation, lymphocytes, monocytes, plasma cell infiltration, necrosis, fibrosis. Conclusions PBC and AIH are two common clinical types of autoimmune liver disease, of which AIH predominates in type I, while AMA-M2 in blood is a characteristic antibody of PBC. Prednisolone treatment is superior to PBC in the treatment of AIH.