新疆地区自身免疫性肝病321例

Autoimmune liver disease in Xinjiang,China:analysis of 321 cases

目的:分析自身免疫性肝病(ALD)临床特征及实验室检测结果,为进一步ALD的诊断和治疗提供理论依据.方法:对321例ALD患者的临床及实验室检查资料进行回顾性分析,比较自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)、原发性硬化性胆管炎(PSC)及自身免疫重叠综合征(OLS)的临床表现、自身抗体的检测、病理特点等.结果:(1)ALD临床表现特点各异,以皮肤瘙痒、肝功异常较多;(2)自身抗体检测:汉、维、其他民族(包括哈萨克、蒙古、回族等)ANA阳性率比较,AIH组差异均无统计学意义(P>0.05),PBC组差异有统计学意义(P<0.05),其他民族(哈萨克、蒙古、回族)ANA阳性率高于汉、维族(70.58%vs40%,47.82%).AMA阳性检测,AIH、PBC组差异均无统计学意义(P>0.05),SMA阳性检测,AIH、PBC组差异均无统计学意义(P>0.05);(3)病理组织:共有96例行肝穿刺病理活检,病理结果显示75.72%(75/96)符合诊断.结论:ALD早期诊断率低,而且常常表现不典型,往往要密切联系临床表现、血液生化及自身抗体检测结果等才能作出正确诊断.

AIM：To analyze the clinical and laboratory characteristics of autoimmune liver disease （ALD） to provide a theoretical basis for diagnosis and treatment of ALD.METHODS：The clinical and laboratory data for 23 ALD patients were retrospectively analyzed.By comparing autoimmune hepatitis （AIH）,primary biliary cirrhosis （PBC）,primary sclerosing cholangitis （PSC） and autoimmune overlap syndrome （OLS） in terms of clinical manifestations,autoantibody detection and pathological features,the clinical and laboratory characteristics of ALD were summarized.RESULTS：ALD patients often showed skin itching and abnormal liver function.The positive rate of antinuclear antibody （ANA） showed no significant difference in AIH patients （P 0.05） but differed significantly in PBC patients （P 0.05） among Han,Uygur and other ethnic groups （including Kazake,Mongolia,Hui,etc.）.The positive rate of ANA was lower in Han and Uygur patients than in other ethnic groups （40% and 47.82% vs 70.58%）.The positive rates of antimitochondrial antibody （AMA） and anti-smooth muscle antibody （SMA） showed no significant differences in either AIH or PBC patients among different ethnic groups （all P 0.05）.A total of 96 patients underwent routine liver puncture biopsy,and 75.72% （75/96） of them were clearly diagnosed.CONCLUSION：The rate of early diagnosis of ALD is very low due to atypical manifestations of the disease.Analysis of clinical manifestations and serum biochemical and autoantibody detection can help diagnose ALD.