135例自身免疫性肝病患者的临床特征分析

Analysis of Clinical Features of 135 Patients with Autoimmune Liver Disease

摘要: 目的：分析自身免疫性肝病(autoimmune liver disease, AILD)患者的临床及影像学特点，明确其最常见分型如自身免疫性肝炎(autoimmune hepatitis, AIH)、原发性胆汁性胆管炎(primary biliary cholangitis, PBC)和AIH-PBC重叠综合征(overlap syndromes, OS)的基本特征，帮助临床及时诊断和治疗。方法：回顾性分析我院2022~2023年收治的135例AILD患者的一般资料，其中PBC组86例，AIH组32例，AIH-PBC OS组17例。结果：三种疾病均好发于中老年女性，临床表现可出现乏力、黄疸、发热等。PBC组碱性磷酸酶(ALP)、白蛋白(ALB)水平高于AIH组；而AIH组丙氨酸氨基转移酶(ALT)、总胆红素(TBIL)水平高于PBC组。PBC组免疫球蛋白IgM的表达水平高于AIH组。ANA在PBC组中的阳性率显著高于AIH组和AIH-PBC OS组，AMA/AMA-M2和抗sp100抗体在PBC组中的阳性率高于AIH组，抗gp210抗体在PBC组和AIH-PBC OS组的阳性率均高于AIH组。PBC组肝硬化、门脉高压、静脉曲张、脾大的发生率高于AIH组，但差异无统计学意义。结论：AILD起病隐匿，无特异性临床表现，容易出现误诊和漏诊。中老年女性如发生不明原因的肝功能损伤，应充分考虑AILD的可能。临床应重视肝功能、免疫球蛋白、自身抗体的检测和影像学检查，以提高该疾病的早期诊断和鉴别能力并改善患者预后。

Abstract: Objective: To analyze the clinical and imaging characteristics of patients with autoimmune liver disease (AILD), clarify the basic features of its most common subtypes such as autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and AIH-PBC overlap syndromes (OS), and assist in timely diagnosis and treatment in clinical practice. Method: General data of 135 AILD patients admitted to our hospital from 2022 to 2023 were retrospectively analyzed, including 86 cases in the PBC group, 32 cases in the AIH group, and 17 cases in the AIH-PBC OS group. Result: All three diseases are prone to occur in middle-aged and elderly women, and the clinical manifestations may include fatigue, jaundice, fever, etc. The levels of alkaline phosphatase (ALP) and albumin (ALB) in the PBC group were higher than those in the AIH group. The levels of alanine aminotransferase (ALT) and total bilirubin (TBIL) in the AIH group were higher than those in the PBC group. The expression level of immunoglobulin IgM in the PBC group was higher than that in the AIH group. The positive rate of ANA in the PBC group was significantly higher than that in the AIH group and the AIH-PBC OS group. The positive rates of AMA/AMA-M2 and anti-sp100 antibody in the PBC group were higher than those in the AIH group, and the positive rates of anti-gp210 antibody in both the PBC group and the AIH-PBC OS group were higher than those in the AIH group. The incidences of liver cirrhosis, portal hypertension, varicose veins and splenomegaly in the PBC group were higher than those in the AIH group, but the difference was not statistically significant. Conclusion: AILD has an insidious onset, no specific clinical manifestations, and is prone to misdiagnosis and missed diagnosis. If middle-aged and elderly women experience unexplained liver function damage, the possibility of AILD should be fully considered. Clinical attention should be paid to the detection of liver function, immunoglobulin, and autoantibodies to improve the early diagnosis and differentiation ability of the disease and improve patient prognosis.