Primary biliary cholangitis (PBC) is an immune-mediated chronic liver disease characterized by biliary epithelial injury, cholestasis, and progressive fibrosis that can lead to cirrhosis and requirement for liver transplantation. All patients with PBC should receive initial treatment with ursodeoxycholic acid (UDCA), and odds for response are based on characteristics at baseline. It is important to have clear definitions of patients at risk for a poor response to therapy, of biochemical markers of an incomplete response, and standardized management. Patients typically are assessed after 12 months of treatment with UDCA for biochemical markers of response. However, evaluation at 6 months has been proposed for patients with more severe disease or symptoms (such as pruritus or fatigue). Markers of response to therapy include reduced serum levels of alkaline phosphatase and bilirubin (Paris-2, Toronto, GLOBE, and so forth); patients with high levels of total and conjugated bilirubin or levels of alkaline phosphatase more than 1.5-fold the upper limit of normal should be considered for second-line therapy. Patients with adequate biochemical responses can continue UDCA monotherapy. Incomplete responders should be considered for second-line therapies with obeticholic acid (licensed) or fibrates (unlicensed) in addition to continued treatment with UDCA. Patients with PBC should be followed up for life.

原发性胆汁性胆管炎 (PBC) 是一种免疫介导的慢性肝病，其特征是胆管上皮损伤、胆汁淤积和进行性纤维化，可导致肝硬化和需要肝移植。所有 PBC 患者都应接受熊去氧胆酸 (UDCA) 的初始治疗，反应几率基于基线特征。对有治疗反应不佳风险的患者、不完全反应的生化标志物和标准化管理有明确的定义是很重要的。通常在用 UDCA 治疗 12 个月后评估患者的生化反应标志物。然而，已建议对疾病或症状更严重（如瘙痒或疲劳）的患者在 6 个月时进行评估。对治疗有反应的标志包括血清碱性磷酸酶和胆红素水平降低（Paris-2、Toronto、GLOBE 等）；总胆红素和结合胆红素水平高或碱性磷酸酶水平超过正常上限 1.5 倍的患者应考虑二线治疗。具有足够生化反应的患者可以继续 UDCA 单药治疗。除了继续使用 UDCA 治疗外，还应考虑对不完全反应者使用奥贝胆酸（已获批）或贝特类（未获批）进行二线治疗。PBC 患者应终生随访。二线治疗应考虑正常上限的 5 倍。具有足够生化反应的患者可以继续 UDCA 单药治疗。除了继续使用 UDCA 治疗外，还应考虑对不完全反应者使用奥贝胆酸（已获批）或贝特类（未获批）进行二线治疗。PBC 患者应终生随访。二线治疗应考虑正常上限的 5 倍。具有足够生化反应的患者可以继续 UDCA 单药治疗。除了继续使用 UDCA 治疗外，还应考虑对不完全反应者使用奥贝胆酸（已获批）或贝特类（未获批）进行二线治疗。PBC 患者应终生随访。