自身免疫性肝炎19例临床分析

CLINICAL ANALYSIS OF 19 CASES OF AUTOIMMUNE HEPATITIS

[目的]分析自身免疫性肝炎的临床和病理特点,为本病的早期诊断、治疗提供可靠的依据。[方法]回顾性分析19例自身免疫性肝炎的一般资料、临床表现、生化及自身抗体特点、病理变化、治疗反应。[结果]男女比例为1︰3.75,发病年龄高峰在50岁左右,15例首诊为病毒性肝炎和/或肝硬化,临床表现以乏力、食欲不振、黄疸、发热、尿黄为主。本组患者临床表现不典型,肝功能各项指标普遍异常,以丙氨酸氨基转移酶、门冬氨酸氨基转移酶、球蛋白升高更为明显。检出的自身抗体分别为:抗核杭体(68.4%),抗平滑肌抗体(31.6%),抗线粒体抗体(21.1%),抗肝肾微粒体1型抗体(5.3%),抗可溶性抗原/肝胰抗原抗体(10.5%)。合并肝外自身免疫性疾病占21.1%。7例患者进行了病理组织学检查,肝组织病理变化以界面炎为主,严重病例则出现小叶性肝炎、桥接坏死、玫瑰花结样改变。合理应用免疫抑制剂可使76.5%AIH患者的肝功能和主要症状在3个月后得到明显改善。[结论]自身免疫性肝炎好发于女性,临床表现不典型,以Ⅰ型AIH最常见,主要检出ANA和/或SMA,患者常伴发肝外自身免疫性疾病;肝组织学损害以界面炎为主;可通过自身抗体的检测结合临床特点获得诊断;免疫抑制治疗有效。

[Objective]To analyze clinical and pathologic characteristics of autoimmune hepatitis（AIH）,and provide basis for early diagnosis and therapy of AIH.[Methods]A retrospective study was conducted to analyze the general information,clinical manifestations,biochemical and immunological features,pathological changes,therapeutic reaction of 19 patients with AIH.[Results]The male female ratio（M-F）was 1：3.75.The age of onset was approximately 50 years.15 patients with AIH were first diagnosed viral hepatitis and/or cirrhosis,and the main clinical manifestations were weakness,anorexia,jaundice,fever and yellow urine.Patients in our study presented with atypical manifestations and all indexes of the liver function showed abnormality especially obviously increased among following indexes：Alanine aminotransferase（ALT）,aspartate aminotransferase（AST）and globulin（GLB）.The positive rates of anti-nuclear antibody（ANA）,anti-smooth muscle antibody（SMA）,anti-mitochondria antibody（AMA）,anti-liver kidney microsomal antibody type 1（LKM-1）and anti-soluble liver antigen/liver pancreas antigen（SLA/LP）were 68.4%,31.6%,21.1%,5.3% and 10.5%,respectively.Patients complicated with autoimmune diseases out of liver accounted for 21.1%.7 patients were conducted histopathologic examinations,and the pathological changes of their hepatic tissue mainly presented with interface hepatitis,while lobular hepatitis,bridging necrosis and rosette of liver cells were observed in heavy patients.Among 76.5% of the patients,the hepatic function and cardinal symptoms could be obviously improved after 3 months by reasonable immunosuppressive therapy.[Conclusion]AIH patients are mainly female,which usually present with atypical manifestations.The main type of AIH is TypeⅠ,and the main detectable antibodies are ANA and AMA;AIH patients usually complicate with autoimmune diseases out of liver;the pathological changes of their hepatic tissue mainly present with interface hepatitis;AIH could be diagnosed by combination of autoantibody detection and clinical manifestations;Immunosuppressive therapy is effective on AIH.