自身免疫性胆管炎与原发性胆汁性肝硬化的临床病理比较

Comparison of the clinical and pathologic characteristics of autoimmune cholangitis and primary biliary cirrhosis

目的分析比较自身免疫性胆管炎与原发性胆汁性肝硬化的临床表现、血清学和病理学特点,探讨自身免疫性胆管炎的临床特征。方法回顾性分析1998年3月至2006年12月中日友好医院门诊和住院的原发性胆汁性肝硬化患者的临床病理资料。结果自身免疫性胆管炎(AIC)与原发性胆汁性肝硬化(PBC)发病均以中老年女性为主,但AIC发病平均年龄较PBC年轻约10岁,而且各种临床症状及体征均较PBC轻;AIC患者血清γ-球蛋白及血清雌二醇水平均较PBC高,而IgM水平较低;免疫学指标AIC以ANA阳性为主(79.59%),PBC患者以AMA阳性为主(86.67%);病理学检查AIC可见胆管缺失或损伤伴有汇管区的炎症和坏死,还可见细小胆管增生,PBC可见间隔或桥接纤维化或再生结节。结论自身免疫性胆管炎是自身免疫性肝病的一个组成部分,可能是PBC的一个发展阶段或者是仅以胆管损伤为主的独立疾病,病情相对较轻。

Objective To compare the clinical and pathologic characteristics of autoimmune cholangitis(AIC) and primary biliary cirrhosis(PBC) and to investigate the clinical feature of autoimmune cholangitis(AIC).Methods Data of 79 cases with primary biliary cirrhosis(PBC) from 1998 to 2006 were retrospectively reviewed.Results Autoimmune cholangitis and primary biliary cirrhosis mainly involved women in 40-60 years old,but the patients with AIC were about 10-year-younger than those with PBC.A variety of clinical symptoms and physical signs were less serious in patients with AIC than PBC.The level of serum gamma globulin and estradiol of patients with AIC was higher than those with PBC,while the level of IgM was lower than that of patients with PBC.Antibodies test showed 79.59 % patients with AIC presented significant titer of antinuclear antibodies(ANA),while 86.67 % patients with PBC presented AMA/AMA-M2.Hepatic histology examination showed ductal and periductal inflammation with cholangiolar proliferation in patients with AIC,while periportal and septal fibrosis or cirrhosis in patients with PBC.Conclusions AIC may be a part of autoimmune liver disease(ALD) with relatively slighter degree.Perhaps it is the early stage of primary biliary cirrhosis or a distinct entity mainly with ductal injury.