35例自身免疫性肝病的临床特点与分析

Comparison and analysis of the clinical characteristics of 35 cases autoimmune liver disease

目的分析比较三种自身免疫性肝病(ALD)的临床表现、血清学和病理学特点及诊断意义。方法回顾性总结昆明医学院第二附属医院2003～2005年35例自身免疫性肝病患者的临床资料。结果①临床表现:ALD以中年女性多见,自身免疫性肝炎(AIH)和原发性硬化性胆管炎(PSC)在年龄上有显著差异,PSC的发热症状较AIH和原发性胆汁性肝硬化(PBC)明显,常伴有胆系感染;②实验室检查:AIH表现为球蛋白显著升高,同时AST、ALT及ALP、GGT升高显著,PSC的TB和DB水平最高,IgG在AIH、PSC中的浓度与PBC比较有显著性差异,明显增高。同时IgA在PSC中浓度与AIH、PBC比较有显著性差异,其浓度明显高于其它两种ALD,AIH患者ANA阳性检出率为75%,可能存在AIHPSC重叠综合症,其发生率约占17.6%;③影像学检查:PSC患者100%有肝内、外胆管的僵硬、狭窄、变细或局部扩张;④病理学:共有6例行肝穿刺病理活检,病理结果提出明确诊断者2例(33.3%)。结论ALD临床表现复杂多样但各有特点,血清免疫学及抗体检测有助于AIH与PBC的诊断和鉴别诊断,ERCP或MRCP有助于PSC的确诊,病理学检查的诊断价值有一定的局限性。

Objective To compare the clinical, serological and pathological characteristics of different autoimmune liver disease （ALD）.Methods The date of 35 cases with ALD from 2003 to 2005 were retrospectively reviewed.Results 1. Clinical features： ALD affected middle-aged women predominantly. Fever in PSC which was accompanied with biliary infection was more common than that in AIH . 2. Laboratory tests ： The abnormality of liver function test could be seen in all of the cases. AIH was characterized by high globulin, alanine aminotransferase（ALT）, aspartate aminotransferase（AST）, alkaline phosphatase（ALP） and γ-glutamyltransferase（γ-GGT）. Total bilirubin（TB） and direct bilirubin （DB） in PSC was highest than that in other two ALD. Immunoglobulin G in AIH and PSC was highest than that in PBC and immunoglobulin A in PSC was highest than that in AIH and PBC.75% of patients with AIH presented significant titers of antinuclear antibody（ANA）, and 17.6% of patients with AIH-PSC overlap syndrome may occur in ALD. 3.Cholangiography： Endoscopic retrograde cholangiopancreatography （ERCP） or magnetic resonance cholangiopancreatography（MRCP） showed irregular stenoses and dilation of the common bile duct or intrahepatic ducts in PSC only .4. Hepatic histology： The histological diagnosis of ALD was made in only 33.3 % （2/6） of the patients with the clinical diagnosis. Conclusion The three kinds of ALD have their relatively specific features. Serum immunology and autoantibodies such as ANA, SMA, especially AMA-M2 are important for diagnosis and differential diagnosis of AIH and PBC. Cholangiography is usually required for conforming a diagnosis of PSC . The significance of liver biopsy in the diagnosis is limited.