摘要
目的分析比较自身免疫性肝炎(autoimmune hepatitis,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis)及其重叠综合征的临床特点、生化特征和治疗反应,提高对自身免疫性肝病的认识。方法对77例AIH患者、46例PBC患者、11例PSC患者和30例PBC-AIH重叠综合征患者的临床及实验室检查资料进行回顾性分析。结果除PSC外,大多数自身免疫性肝病多发于中年女性,从出现症状到明确诊断平均需要2.5年。AIH、PBC-AIH重叠患者具有较高的转氨酶,PBC、PSC具有较明显的GGT、ALP升高。临床表现上AIH、PBC、PSC、AIH-PBC黄疸发生率分别为84%、78%、90%和67%,皮肤瘙痒的发生率分别为43%、56%、81%和60%。PSC和AIH-PBC具有较高的AIH评分,27%的PSC患者和33%AIH-PBC的评分达到可能的AIH。合理应用UDCA和免疫抑制剂可使90%的PBC和AIH患者症状在六个月内得到缓解、肝功能恢复明显改善。结论AIH、PBC-AIH的肝功能异常以转氨酶升高为主,PBC、PSC以胆汁淤积为主。应用AIH评分系统诊断可能的AIH时应注意鉴别PSC及其它自身免疫性肝病。UDCA和免疫抑制剂可改善绝大多数患者的症状和肝功能异常。
Objective To analize and compare the clinical characteristics of AIH, PBC, PSC and PBC - AIH overlap syndrome. Methods Total RNAs were extracted from circulating blood, synthesized to cDNA through random primers and reverse transcriptase, and the gene fragments of AFP were amplified by a nested-polymerase chain reaction （nested-PCR） assay. AFP-L3 was separated by lectin-affinity chromatography and both of AFP and AFP-L3 were detected by radio- immunoassay （RIA）. Results The clinical manifestation, laboratory test and the response to therapy of 77 cases of AIH,46 cases of PBC, 11 cases of PSC and 30 cases of PBC-AIH overlap syndrome were investigated retrospectively. Results Except for PSC, the autoimmune hepatic diseases most frequently affected middle-aged women, the mean time interval between the first visit to physicians to the time of correct diagnosis was over 2.5 years. ALT and AST levels were markedly elevated in patients with AIH and AIH - PBC overlap syndrome , whereas ALP and GGT levels were significantly elevated in patients with PBC and PSC. The incidences of jaundice in AIH, PBC, PSC and PBC - AIH overlap syndrome were 84 %, 78 %, 90 % and 67 % respectively, and those of pruritus were 43 %, 56 %, 81% and 60 % respectively. Twenty seven percent patients of PSC and 33 % patients AIH - PBC could be diagnosed as probable AIH by using the scoring system of AIH. UDCA and immunosuppressive therapy could attain a marked biochemical and clinical improvement in most patients within six months. Conclusion AIH and PBC - AIH overlap syndrome have servere liver impairment, whereas liver function tests of PBC and PSC typically reveal a cholestatic pattern. PSC and other autoimmune hepatic diseases should be carefully excluded when we use the the scoring system of AIH. UDCA and immunosuppressive therapy could improve most of the abnormal liver function tests and clinical features.
出处
《胃肠病学和肝病学杂志》
CAS
2006年第3期274-276,280,共4页
Chinese Journal of Gastroenterology and Hepatology
基金
南通市社会发展科技计划(s30033)
关键词
肝硬化
临床医学
肝炎
自身免疫性
原发性硬化性胆管炎
Liver Cirrhosis
Clinical Medicine
Hepatitis, Autoimmune
Primary Sclerosing Cholangitis
