自身免疫性肝病临床与病理研究

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。

Clinical and pathological study of autoimmune hepatopathy

Objective To study the clinical and histologic characteristics of autoimmune hepatopathy, and to explore the diagnostic criteria and therapy. Methods Sixty-two patients with autoimmune hepatopathy were studied between 1996 and 1998 to compare primary binary cirrhosis (PBC) and autoimmune hepatitis (AIH) in the clinical symptoms, signs, laboratory findings and histologic features. Results PBC was 59.68% and AIH 40.32% in the autoimmune hepatopathy. The morbidity in middle and old aged female patients with autoimmune hepatopathy was 83.8%. The ratio of male and female was l:10. Whereas, the ratio of male and female was l:5 in AIH patients and average age was 35 years old. Seropositive autoimmune antibodies were detected in 53 serum sample (PBC 85.29%; AIH 78.95%). Positive rates of the mitochondrial antibody and subtype-MZ were 100% for PBC. IgM increasing was 80% in PBC (P<0.05) and 82.35% in AIH (P<0.025). Histologic features in PBC group consisted of cholangitis, destruction or proliferation of the bile duct and inflammation within the portal tract; while in AIH group inflammation, necrosis and fibrosis of periportal region, the aggregation of lymphocytes, mononuclear cells and plasma cells in the portal tract or periportal area were found. Conclusions PBC and AIH are the most common two types in the patients with autoimmune hepatopathy. AIH-I is more often seen clinically. AHA-M2 is the special autoimmune antibody of PBC. The efficacy of prednisolone is better for AIH than PBC.