不明原因肝功能异常患者中自身免疫性肝病的临床分析

背景:自身免疫性肝病(AILD)起病隐匿,临床表现无特异性,早期诊断十分困难。目的:总结不明原因肝功能异常患者随访过程中AILD的诊断情况。方法:回顾性分析161例不明原因肝功能异常患者的临床随访结果,包括AILD相关自身抗体监测,部分患者经本人同意后行肝穿刺活检病理检查。结果:在6个月～6年的随访中,57例患者诊断为AILD,其中自身免疫性肝炎(AIH)28例,原发性胆汁性肝硬化(PBC)21例,PBC-AIH重叠综合征8例。初次入院时,AILD患者临床表现与非AILD患者无明显差异,但无症状者所占比例较大;血生化指标中的GGT、ALP、球蛋白水平、免疫相关指标中的IgG、IgM水平均显著高于非AILD患者。AILD患者自身抗体ANA、AMA/AMA-M2、SMA阳性率显著高于非AILD患者,肝活检病理检查结果与相应疾病的组织病理学改变相符。结论:对于不明原因的反复肝功能异常患者,定期监测AILD相关自身抗体和肝活检病理有助于早期诊断、早期治疗AILD。

Autoimmune Liver Disease in Patients with Liver Dysfunction of Unknown Etiology: A Clinical Analysis

Background： It is difficult to diagnose autoimmune liver disease （AILD） at early stage because of its insidious onset and nonspeeific clinical features. Aims： To analyze the diagnosis of AILD in patients with liver dysfunction of unknown etiology during the follow-up period. Methods： The follow-up outcomes of 161 patients with liver dysfunction of unknown etiology were studied retrospectively. During the follow-up period, AILD-related autoantibodies were monitored, and liver biopsy pathology was studied with informed consent from the patients. Results： Fifty-seven patients were diagnosed as AILD during 6 months to 6 years follow-up, including 28 autoimmune hepatitis （AIH）, 21 primary biliary cirrhosis （PBC）, and 8 PBC-AIH overlap syndrome. When compared with non-AILD patients, the clinical features of AILD patients on admission were similar, but there were more asymptomatic cases, and with higher serum levels of GGT, ALP, globulin, IgG and IgM. AILD patients had higher positivity rates of AILD-related autoantibodies, such as ANA, AMA/AMA-M2, and SMA. The findings of liver biopsy pathology fulfilled the histopathological features of AIH, PBC, or PBC-AIH overlap syndrome. Conclusions： AILD-related autoantibodies and liver biopsy pathology should be monitored at regular intervals in patients with liver dysfunction of unknown etiology. It will be helpful for diagnosing and treating AILD patients at early stage.