Ⅰ型自身免疫性肝炎患者的临床病理分析  

Clinicopathological features of patients with autoimmune hepatitis type Ⅰ

作　　者：邱德凯[1] 马雄[1] 

机构地区：[1]上海第二医科大学仁济医院,上海市消化疾病研究所200001

出　　处：《中华消化杂志》2002年第5期273-278,I001,共7页Chinese Journal of Digestion

摘　　要：目的　从病毒标志物均阴性的肝功能异常患者中筛选Ⅰ型自身免疫性肝炎 (AIH)病例 ,并探讨其临床病理表现特征。方法　采用国际自身免疫性肝炎小组 (IAIHG)新修订的描述性诊断标准和计分系统 ,对病毒标志物均阴性的肝功能异常患者进行筛选 ,对符合诊断的患者进行临床病理特点分析。结果　符合诊断的 32例Ⅰ型AIH患者均为女性 ,高发年龄为 4 0～ 6 0岁 ,肝功能生化检查表现为肝炎样改变。 2 8例 (87.5 % )表现为高γ 球蛋白血症 ,2 6例 (81.3% )IgG高于正常。 2 6例 (81.3% )抗核抗体 (ANA)阳性。伴发相关的自身免疫性疾病 12例 (37.5 % )。在有肝穿刺活检组织资料的 15例患者中 ,11例 (73.3% )出现界板性肝炎 ,小叶性肝炎 5例 (33.3% ) ,玫瑰花结样改变 3例 (2 0 .0 % ) ,肝纤维化或肝硬化 6例 (40 .0 % )。 2 1例 (6 5 .6 % )患者对免疫抑制治疗完全应答 ,9例 (2 8.1% )出现复发 ,12例(37.5 % )持续缓解。结论　Ⅰ型AIH患者多为中年女性 ,以肝组织活检呈界板性肝炎、血清自身抗体、高γObjective We screened autoimmune hepatitis (AIH) from patients who had abnormal liver function without any viral marker in serum, and investigated the clinical and pathological features of AIH type Ⅰ. Methods According to the revised descriptive criteria and scoring system for diagnosis of autoimmune hepatitis proposed by the International Autoimmune Hepatitis Group, we examined the clinical and pathological characteristics of the patients who were diagnosed as AIH type Ⅰ. Results All patients (32 cases) diagnosed as AIH type Ⅰ were female. The age distribution showed a single peak in the decades of the fifties and sixties. Liver function test of these patients showed hepatic profile. Twenty eight patients (87.5%) had hypergammaglobulinemia, 26(81.3%) and elevated serum IgG level. Twenty six cases were positive for antinuclear antibody (ANA). Twelve patients (37.5%) had concurrent autoimmune diseases. Among fifteen patients who underwent liver biopsy, interface hepatitis and lobular hepatitis were observed in 11(73.3%) and 5(33.3%) patients, respectively. Rosetting of liver cells and liver fibrosis or cirrhosis were also detected in 3(20%) and 6(40%) patients respectively. Twenty one patients (65.5%) were considered to have a complete response to immunosuppressive therapy; 9(28.1%) relapsed; and 12(37.5%) sustained remission. Conclusions Autoimmune hepatitis type Ⅰ is mainly diagnosed in middle aged women, characterized by interface hepatitis on histologic examination, the presence of circulating autoantibody, hypergammaglobulinemia and response to immunosupressive treatment.

关 键 词：I型自身免疫性肝炎 临床病理 诊断 治疗 

分 类 号：R575.1[医药卫生—消化系统]