自身抗体阴性自身免疫性肝炎患者临床病理学特点

Clinical and Histological Features of Autoantibody-negative Autoimmune Hepatitis in Chinese Patients:A Single Center Experience

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.