【摘要】 目的 比较药物与自身免疫性肝病所致胆管消失综合征（VBDS）患者的临床病理特征及预后差异，总结临床鉴别要点。方法 选取2018年1月—2024年6月在苏州大学附属第一医院、无锡市第五人民医院经皮肝脏活组织检查明确诊断为VBDS的67例患者为研究对象，其中药物所致VBDS(D-VBDS)18例，自身免疫性肝病所致VBDS(A-VBDS)49例。收集患者的一般资料、临床症状及体征、实验室指标、肝脏病理形态学特征及预后情况。符合正态分布的计量资料两组间比较采用成组t检验，非正态分布的计量资料两组间比较采用Mann-Whitney U秩和检验；计数资料两组间比较采用χ~2检验或Fisher’s精确检验。结果 67例VBDS患者中女性占比较多（80.60%），年龄以40～59岁为主（55.22%）。其中18例（26.87%）由药物引起，49例（73.13%）由自身免疫性肝病引起。VBDS患者常见的临床症状依次为尿黄（83.58%）、乏力（64.18%）、纳差（64.18%）、巩膜黄染（62.69%）、腹胀（23.88%）、皮肤瘙痒（23.88%）、恶心呕吐（20.90%）和发热（13.43%）。与D-VBDS组相比，A-VBDS组患者浆细胞浸润程度更高，毛细胆管胆栓程度更低，差异均有统计学意义（χ~2值分别为14.186、6.568,P值均<0.05）。D-VBDS组患者的丙氨酸氨基转移酶、总胆红素、直接胆红素和间接胆红素峰值明显高于A-VBDS组，差异均有统计学意义（Z值分别为-2.546、-2.957、-2.628、-2.772,P值均<0.05）。与D-VBDS组相比，A-VBDS组患者更易发生肝硬化，差异有统计学意义（χ~2=4.682,P=0.030）。结论 丙氨酸氨基转移酶、胆红素水平以及肝组织病理形态特点是鉴别D-VBDS和A-VBDS的客观指标，对明确诊断、判断病变损伤程度具有重要意义。自身免疫性肝病所致VBDS患者更易发展为肝硬化。更多还原

【Abstract】 Objective To investigate the differences in clinicopathological features and prognosis between patients with druginduced vanishing bile duct syndrome(VBDS) and those with VBDS induced by autoimmune liver disease, and to summarize the key points for clinical differentiation. Methods A total of 67 patients who were diagnosed with VBDS by liver biopsy in The First Affiliated Hospital of Soochow University and Wuxi Fifth People’s Hospital from January 2018 to June 2024 were enrolled as subjects, among whom 18 had drug-induced VBDS(D-VBDS group) and 49 had VBDS induced by autoimmune liver disease(A-VBDS group). Related data were collected, including general information, clinical symptoms and signs, laboratory markers, liver pathomorphological features, and prognosis. The independent-samples t test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups; the chi-square test or the Fisher’s exact test was used for comparison of categorical data between two groups. Results Among the 67 VBDS patients, female patients accounted for a higher proportion(80.60%), and the patients aged 40 — 59 years accounted for 55.22%; there were 18 patients(26.87%) with D-VBDS and 49 patients(73.13%) with A-VBDS. Yellow urine(83.58%) was the most common clinical symptom in VBDS patients, followed by fatigue(64.18%), poor appetite(64.18%), jaundice of the sclera(62.69%), abdominal distension(23.88%), pruritus(23.88%), nausea and vomiting(20.90%), and fever(13.43%). Compared with the D-VBDS group, the A-VBDS group had a significantly higher degree of plasma cell infiltration and a significantly lower degree of canalicular bile plugs （χ~2=14.186, 6.568, both P<0.05）. Compared with the A-VBDS group, the D-VBDS group had significantly higher peak levels of alanine aminotransferase, total bilirubin, direct bilirubin, and indirect bilirubin(Z=-2.546,-2.957,-2.628,-2.772, all P<0.05). Compared with the D-VBDS group, the patients in the A-VBDS group were more likely to develop liver cirrhosis （χ~2=4.682, P=0.030）. Conclusion The levels of alanine aminotransferase and bilirubin and liver pathomorphological features are objective indicators for differentiating D-VBDS from A-VBDS, and they are of great importance for clarifying diagnosis and determining the degree of liver injury. Patients with A-VBDS are more likely to develop liver cirrhosis.更多还原