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Guidelines on the diagnosis and management of primary biliary cholangitis (2021)
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摘要: 2015年中华医学会肝病学分会和中华医学会消化病学分会制订了我国第一个原发性胆汁性胆管炎(primary biliary cholangitis, PBC)的专家共识。近年来国内外有关研究为PBC的诊断和治疗提供了新的临床证据。中华医学会肝病学分会组织有关专家, 在评估最新国内外临床研究证据的基础上, 制订了包括26条推荐意见的本指南, 旨在为临床医师诊断和治疗PBC提供指导和参考意见。Abstract: In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued the consensus on the diagnosis and management of primary biliary cholangitis (PBC). In the past years, more clinical studies have been reported in the field of PBC. To provide guidance to the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology invited a panel of experts assessed the new clinical evidence and formulated the current guidelines which comprises 26 clinical recommendations.
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Key words:
- Primary Biliary Cholangitis /
- Primary Biliary Cirrhosis /
- Diagnosis /
- Therapeutics /
- Practice Guideline
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表 1 推荐意见的证据质量和推荐强度分级
级别 详细说明 证据质量 高(A) 进一步研究不可能改变对该疗效评估结果的可信度 中(B) 进一步研究有可能影响该疗效评估结果的可信度, 且可能改变该评估结果 低或非常低(C) 进一步研究很有可能影响该疗效评估结果的可信度, 且很可能改变该评估结果 推荐强度 强(1级) 明确显示干预措施利大于弊或者弊大于利 弱(2级) 利弊不确定或无论质量高低的证据均显示利弊相当 
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表 2 PBC的Ludwig分期
Ludwig分期 组织学特点 Ⅰ期：胆管炎期 炎症局限于汇管区, 受损的小胆管周围以淋巴、单核细胞浸润为主, 亦可见浆细胞、嗜酸性粒细胞及少数中性粒细胞, 有的胆管周围可见非干酪性肉芽肿, 即特征性的旺炽性胆管病变(florid duct lesion) Ⅱ期：汇管区周围炎期 汇管区炎症可突破界板深入小叶内, 同时汇管区周边带可见细胆管增生, 形成胆管性界面炎 Ⅲ：进行性纤维化期 部分纤维化扩大的汇管区之间以桥接纤维间隔相连 Ⅳ：肝硬化期 汇管区之间的桥接纤维间隔分隔肝实质呈“七巧板”图样
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表 3 PBC的生化应答标准
应答标准 时间 定义 巴塞罗那标准 12个月 ALP下降40%或恢复正常 梅奥标准 6个月 ALP＜2×ULN 巴黎Ⅰ标准 12个月 ALP＜3×ULN, AST＜2×ULN和胆红素＜1 mg/dL 巴黎Ⅱ标准 12个月 ALP＜1.5×ULN, AST＜1.5×ULN和胆红素＜1 mg/dL 多伦多标准 24个月 ALP＜1.67×ULN 鹿特丹标准 12个月 胆红素, 白蛋白正常 UK-PBC评分 12个月 基线白蛋白, 血小板；UDCA治疗1年后的胆红素, ALP, ALT或AST；预测5年、10年、15年生存率及对UDCA的应答 PBC GLOBE评分 12个月 基线年龄, UDCA治疗1年后的胆红素, ALP, 白蛋白, 血小板；预测3年, 5年, 10年生存率及对UDCA的应答
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