原發性膽汁性膽管炎之臨床表徵、治療與預後

10.6666/ClinMed.202005_85(5).0053

李文雅；蘇建維

原發性膽汁性膽管炎（primary biliary cholangitis, PBC） ； 熊去氧膽酸（ursodeoxycholic acid, UDCA） ； 奧貝膽酸（obeticholic acid, OCA）

臨床醫學月刊

85卷5期（2020 / 05 / 29）

286 - 291

繁體中文

原發性膽汁性膽管炎（primary biliary cholangitis, PBC）是自體免疫反應導致膽汁滯留的慢性肝病，隨著發炎反應和纖維化的進程，可能發展至肝硬化。此病好發於中年，男女比例為1比9，且較盛行於北歐、北美地區，目前認為由基因遺傳與環境因素共同導致。主要臨床症狀包括倦怠和搔癢。約有一半的患者診斷時無症狀，血清學表現以鹼性磷酸酶（alkaline phosphatase）上升、抗粒線體抗體（anti-mitochondrial antibody）陽性為特色，必要時可採行肝臟切片進行診斷。從1990年代使用的第一線治療藥物熊去氧膽酸（ursodeoxycholic acid, UDCA）至今仍具有十足的證據力，治療一年後以鹼性磷酸酶作為療效評估指標，近年來新的GLOBE score與UK-PBC score等模型，也可作為治療反應與預後的參考；2016年5月美國食品藥品管理局（Food and Drug Administration, FDA）正式核准了奧貝膽酸（obeticholic acid, OCA）作為二線治療，使原發性膽汁性膽管炎治療邁出新的一步。

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