摘要
目的:分析自身抗体阴性自身免疫性肝炎(AIH)患者临床特点,并总结诊疗经验。方法:对167例AIH患者的临床和病理特点进行分析,将自身抗体阴性AIH患者定义为:符合国际自身免疫性肝炎小组(IAIHG)1999年描述性诊断标准且抗核抗体、抗平滑肌抗体、抗肝肾微粒体1型抗体和抗线粒体抗体均为阴性者。结果:167例中17例自身抗体阴性,占10.2%。自身抗体阳性和自身抗体阴性AIH患者在一般情况(诊断时年龄除外)、生化指标方面均无明显差异(P>0.05)。自身抗体阴性组血清IgG水平低于自身抗体阳性组,差异有统计学意义(P=0.004)。两组肝组织学炎症分级无明显差异,而自身抗体阴性组中存在更多纤维化进展期患者(P<0.001)。采用1999年诊断积分系统评估时,自身抗体阴性组中11例(64.7%)为可能性诊断,其余6例(35.3%)为确定性诊断;采用简化诊断积分系统重新评估后,仅3例(17.6%)达到可能性诊断,且无1例达到确定性诊断标准。与自身抗体阳性组相比,自身抗体阴性AIH患者接受24个月的免疫抑制治疗后累积完全生化缓解率为86%,两组患者无明显差异(P=0.658)。结论:自身抗体阴性AIH在国人中并非罕见,在应用免疫抑制治疗方面与自身抗体阳性AIH患者类似,能取得满意疗效。
Aims:To define the clinical features of Chinese patients with autoantibody-negative autoimmune hepatitis (AIH) and to refine the diagnosis and management of these atypical patients in a single Chinese center.Methods:A retrospective evaluation of 167 Chinese patients with AIH was performed.Patients meeting the International Autoimmune Hepatitis Group (IAIHG) comprehensive criteria in 1999 with the absence of antinuclear antibodies,smooth muscle antibodies,liverkidney microsomal-1 antibodies and antimitochondrial antibodies were defined as autoantibody-negative patients.Results:In total,17 (10.2%) of the 167 patients with AIH were autoantibody-negative.Except the age at diagnosis,the general status and biochemical tests between the classical and autoantibody-negative patients were not significantly different (P 〉 0.05).Serum immunoglobulin G levels of the autoantibody-negative AIH patients were lower than those of the classical AIH ones (P =0.004).There was no significant difference in the histological inflammatory grades between the two groups; however,advanced histological stages were more common in the autoantibody-negative AIH group (P 〈0.001).In the autoantibody-negative AIH patients,11 (64.7%) had a possible diagnosis and 6 (35.3 %) had a definite diagnosis according to the comprehensive criteria.While with the simplified criteria only 3 patients (17.6%) had a possible diagnosis,and none had a definite diagnosis of AIH.The complete biochemical remission rate was 86% within 24 months of immunosuppressive therapy,which was comparable to classical AIH (P =0.658).Conclusions:Autoantibodynegative AIH is not uncommon in Chinese AIH patients,and has a good response to immunosuppressive treatment comparable to classical patients.
出处
《胃肠病学》
2014年第3期129-133,共5页
Chinese Journal of Gastroenterology
基金
国家自然科学基金(No.30972751
No.81170380
No.81100271)
2010年上海高校选拔培养优秀青年教师科研专项基金(沪教委人[2010]83号)
关键词
肝炎
自身免疫性
自身抗体
免疫抑制剂
预后
Hepatitis, Autoimmune
Autoantibodies
Immunosuppressive Agents
Prognosis