药物相关性胆管消失综合征的临床病理特征

Clinicopathologic features of drug-induced vanishing bile duct syndrome

胆管消失综合征（VBDS）是由多种因素引起的肝内部分胆管树持续进行性破坏和消失为病理形态学特征，以胆汁淤积为主要临床表现的疾病。其中药物性肝损伤相关的胆管消失综合征（D-VBDS）是VBDS的重要病因之一，免疫紊乱或者免疫失衡可能是主要的发病机制。D-VBDS依据临床症状、血清学指标及病程演变分主要形式及次要形式两种临床类型，其不同的临床症状及病情演变是以综合多样的病理形态学为基础；其预后转归与胆管细胞的再生情况、胆管损伤数量、胆管损伤级别、胆管损伤波及范围、细胆管增生以及其他胆管分支代偿分流等替代途径多种综合因素有关。该病的临床结局呈多样性，多数患者停药之后预后较好，少数则导致胆汁淤积性肝硬化、肝衰竭及死亡。由于其临床表现及生物化学指标改变与原发性胆汁性胆管炎、原发性硬化性胆管炎相类似，需要临床医生予以鉴别。

Vanishing bile duct syndrome （VBDS） manifests as progressive destruction and disappearance of the intrahepatic bile duct caused by various factors and cholestasis. VBDS associated with drug-induced liver injury （D-VBDS） is an important etiology of VBDS, and immune disorder or immune imbalance may be the main pathogenesis. According to its clinical symptoms, serological markers, and course of the disease, D-VBDS is classified into major form and minor form, and its clinical features are based on various pathomorphological findings. Its prognosis is associated various factors including regeneration of bile duct cells, number of bile duct injuries, level and range of bile duct injury, bile duct proliferation, and compensatory shunt of bile duct branches. This disease has various clinical outcomes; most patients have good prognosis after drug withdrawal, and some patients may experience cholestatic cirrhosis, liver failure, and even death. Due to the clinical manifestation and biochemical changes are similar to the primary biliary cholangitis （PBC） and primary sclerosing cholangitis （PSC）, it need to identify by clinical physician.