自身免疫性肝病临床病理特征分析28例  

Clinicopathological features of autoimmune hepatic diseases: an analysis of 28 cases

作　　者：张会爱[1] 王邦茂[1] 章明放[2] 常毅湘[1] 弓艳霞[3] 

机构地区：[1]天津医科大学总医院消化科,天津市300052 [2]天津医科大学总医院病理科,天津市300052 [3]天津市南开医院,天津市300100

出　　处：《世界华人消化杂志》2008年第29期3338-3342,共5页World Chinese Journal of Digestology

摘　　要：目的:探讨并比较自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)及其重叠综合征(AIH-PBCOS)的临床、生化、影象学以及组织学特征.方法:收集在我院自身免疫性肝病住院患者28例,其中I型AIH13例、PBC6例和AIH-PBCOS9例,回顾性分析患者临床资料.结果:AIH-PBCOS患者血清谷草转氨酶(AST)和γ-球蛋白(GLO)水平(分别为和)均明显高于AIH和PBC患者(132.67±35.05vs81.15±39.26,55.17±24.38;44.33±8.92vs34.23±9.89,33.17±6.79,均P<0.05),而碱性磷酸酶(ALP)和γ-谷氨酰转肽酶(GGT)水平明显高于AIH患者(218.11±107.79vs106.85±91.57;280.33±133.51vs82.07±36.67,均P<0.05),低于PBC患者(245.83±88.60,315.67±160.01,均P<0.05).AIH-PBCOS和PBC患者的IgM水平均明显高于AIH患者(793.00±528.57vs127.33±30.14;538.50±349.43vs127.33±30.14,均P<0.05),而AIH-PBCOS患者的IgG水平明显高于PBC患者(2036.00±457.03vs1121.25±313.8,P<0.05).AIH-PBCOS患者界面性肝炎和破坏性胆管炎的发生率分别为88.9%和66.7%.腹部BUS提示三者常伴有脾大和腹腔淋巴结肿大.结论:AIH和PBC是典型的分别选择性损伤肝细胞及肝内小胆管的自身免疫性肝病,AIH-PBCOS同时具有AIH和PBC的临床病理学特征,与PBC相比,AIH-PBCOS更倾向于AIH.AIM： To investigate and compare the clinical, biological, imaging and histological features of patients with autoimmune hepatitis （AIH）, primary biliary cirrhosis （PBC） or AIH-PBC overlap syndrome （AIH-PBC OS）. METHODS： The clinical data and liver biopsy specimens from 13 patients with type I AIH, 6 patients with PBC and 9 patients with AIH-PBC OS in General Hospital of Tianjin Medical University were retrospectively analyzed. RESULTS： Patients with AIH-PBC OS had markedly higher serum aspartate aminotransferase （AST） and y globulin （GLO） levels than patients with AIH or PBC （132.67 ± 35.05 vs 81.15 ± 39.26, 55.17 ± 24.38;44.33 ± 8.92 vs 34.23 ± 9.89, 33.17 ± 6.79, all P 〈 0.05）; serum alkalinephosphatase （ALP） and gammaglutamyl-transpeptidase （GGT） levels were significantly higher in patients with AIH-PBC OS than with AIH （218.11 ± 107.79 vs 106.85 ± 91.57; 280.33 ± 133.51 vs 82.07 ± 36.67, both P 〈 0.05）, but lower than that in patients with PBC （245.83± 88.60, 315.67 ± 160.01, both P 〈 0.05）. IgM levels in patients with PBC or AIH-PBC OS were significantly higher than in patients with AIH （793.00 ± 528.57 vs 127.33 ± 30.14; 538.50 ± 349.43 vs 127.33 ± 30.14, both P 〈 0.05） while patients with AIH-PBC OS had significantly higher IgG levels than patients with PBC （2036.00 ± 457.03 vs 1121.25 ± 313.8, P 〈 0.05）. Histological analysis showed interface hepatitis in 88.9% and destructive cholangitis in 66.7% of AIH-PBC OS patients. Abdominal ultrasound showed that they were all often associated with hypersplenotrophy and celiac lymphadenectasis. CONCLUSION： AIH and PBC are representative autoirnmune liver diseases in which hepatocytes and intrahepatic bile ducts, respectively, are selectively damaged by autoimmune mechanisms. AIH-PBC OS with clinicopathological features of both AIH and PBC shows closer clinically and histologically features to AIH than to PBC.

关 键 词：自身免疫性肝炎 原发性胆汁性肝硬化 重叠综合症 

分 类 号：R575[医药卫生—消化系统] R735.2[医药卫生—内科学]