IgG4相关性Ⅰ型自身免疫性肝炎患者临床特点分析  

Clinical characteristics of patients with IgG4-associated type Ⅰ autoimmune hepatitis

作　　者：刘象 徐芸[1] 汪群英[1] 保洁 吴凤娟[1] 

机构地区：[1]郑州大学第一附属医院消化内一科,450001

出　　处：《中华消化杂志》2014年第8期521-526,共6页Chinese Journal of Digestion

摘　　要：目的 探讨Ⅰ型自身免疫性肝炎（AIH）患者肝脏组织学IgG4的表达,及其临床表现、生物化学指标、免疫及遗传学特性、病理学表现及免疫抑制治疗效果.方法 纳入2012年3月至2013年7月确诊的AIH-Ⅰ型患者45例,对其肝脏组织切片进行CD38、IgG、IgG4免疫组织化学染色,同时进行炎性反应分级G、纤维化分期S,并收集患者相应的血清学指标（ALT、AST、ALP、IgG、抗核抗体效价、去唾液酸糖蛋白受体抗体（ASGPRAb）,血清IgG4测定,进一步研究免疫抑制治疗后的应答反应.定量资料比较采用t检验和Wilcoxon秩和检验,Spearman相关系数表示相关性.结果 45例AIH-Ⅰ型患者中5例（11.1％）为IgG4相关性AIH（IgG4-AIH组）,其与经典AIH组患者（40例）在年龄、性别、生物化学指标（ALT、AST、ALP水平）、免疫指标（IgG水平、抗核抗体效价、ASGPRAb阳性率）及组织纤维化程度等方面差异均无统计学意义（P均＞0.05）.IgG4-AIH组的血清IgG水平为25.8（9.2） mg/L,明显高于经典AIH组的18.3（6.7） mg/L（Z=-2.041,P＜0.05）,但两组之间血清IgG4水平差异并无统计学意义（P＞0.05）,同时可见肝脏组织IgG4浆细胞浸润个数与血清IgG4水平无相关性（r=0.311,P=0.279）.IgG4-AIH组患者的肝脏组织炎性反应情况较经典AIH组更显著（H＝4.120,P＜0.05）,CD38＋、IgG＋浆细胞表达相对较经典AIH组更显著（CD38每个高倍视野下个数分别为39.3（13.5）的和21.3（8.8）,IgG分别为39.3（14.0）和18.5（8.9）;Z＝-3.561,-3.584;P均＜0.01）.肝脏组织中IgG4＋浆细胞个数与CD38＋、IgG＋浆细胞个数呈正相关（r=0.884,0.791;P均＜0.01）.结论 AIH-Ⅰ型患者中存在IgG4相关性AIH,但发病率不高.IgG4相关性AIH患者血清IgG4水平未显著升高,但组织学炎性反应活动较显著,并伴随较多CD38＋、IgG-浆细胞浸润.Objective To investigate the expression of IgG4 in liver tissues of patients with type Ⅰ autoimmune hepatitis （AIH） and to analyze the clinical manifestation,biochemical indexes,immunological genetic features,pathological characteristics and the effects of immunosuppressive therapy.Methods From March 2012 to July 2013,45 patients diagnosed as type Ⅰ AIH were enrolled.Immunostaining of CD38,IgG and IgG4 in liver tissue slices was performed,inflammation grade G and fibrosis stage S were determined.At the same time,serological indexes （alanine aminotransferase （ALT）,aspartate transaminase （AST）,alkaline phosphatase （ALP）,IgG,antinuclear antibody （ANA） titer,antibodies to asialoglycoprotein receptor （ASGPRAb）） of the patients were collected,serum IgG4 was tested and the response to immunosuppressive therapy was observed.Wilcoxon rank sum test and t test were performed for quantitative data comparison.Spearman correlation coefficient was used for correlation analysis.Results Among 45 patients with type Ⅰ AIH,five patients （11.1％） were IgG4 associated AIH （IgG4-AIH group）.There were no significant differences in age,gender,biochemical indexes （serum levels of ALT,AST,ALP）,immunity indexes （serum levels of IgG,ANA titer,ASGPRAb） and degree of liver fibrosis between patients with IgG4-associated AIH and classical AIH （40 cases） （all P＞ 0.05）.Compared with classical AIH group （18.3（6.7） mg/L）.The serum level of IgG4 of IgG4 AIH group was 25.8（9.2） mg/L,which significantly increased （Z=-2.041,P＜0.05）.However,there was no significant difference in serum level of IgG4 between the two groups （P＞0.05）.There was no correlation between the number of infiltrated IgG4 positive plasma cells and the serum level of IgG4 （r=0.311,P=0.279）.The inflammation in the liver tissues of IgG4-AIH group was more significant compared with that of classic AIH group （H=4.120,P＜0.05）.The number of CD38＇ or IgG＋ plasma cells was larger compared with t

关 键 词：肝炎 自身免疫性 IGG4 IGG4相关性疾病 病理学 临床 

分 类 号：R575.1[医药卫生—消化系统]