Development of autoimmune hepatitis in primary biliary cirrhosis
Development of autoimmune hepatitis in primary biliary cirrhosis
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DOI：
10.1111/j.1478-3231.2007.01538.x
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发表时间：
2007-10-01
影响因子：
6.7
通讯作者：
Lindor, Keith D.
中科院分区：
文献类型：
--
作者：
Gossard, Andrea A.;Lindor, Keith D.
目的/背景：原发性胆汁性肝硬化（PBC）是一种病因不明的慢性胆汁淤积性肝病。高达10%具有PBC典型特征的患者会伴有自身免疫性肝炎（AIH）的附加特征。然而，一个子集没有这些特征，但继续发展为“顺序”AIH重叠综合征。目的：描述我们在诊断PBC后发展为AIH的8例患者的经验。方法：我们回顾了1996年至2005年9年间所有PBC患者的图表。仅包括无AIH特征的PBC患者。结果：共1476例PBC患者。其中8例患者根据生化和组织学参数表现出AIH重叠综合征的特征。治疗包括强的松和硫唑嘌呤24个月或更长时间。大多数患者在整个治疗过程中仍然使用熊去氧胆酸（UDCA）。对治疗的反应是通过酶的改善来定义的，并且对所有患者都是快速的。1名患者能够停止强的松和硫唑嘌呤治疗，而7名患者至今仍在继续治疗。结论：AIH与PBC可发生“顺序”重叠综合征。用强的松和硫唑嘌呤治疗可导致转氨酶水平的快速改善。
Aim/Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown aetiology. Up to 10% of patients with typical features of PBC will have additional features of autoimmune hepatitis (AIH). A subset, however, have no such features but go on to develop a 'sequential' AIH overlap syndrome. Objectives: Describe our experience with eight patients who developed AIH after the diagnosis of PBC was made. Methods: We reviewed the charts of all PBC patients over a 9-year period ( from 1996 to 2005). Only PBC patients with no features of AIH were included. Results: There were 1476 patients with PBC. Of these, eight patients developed features of AIH overlap syndrome based on biochemical and histological parameters. Treatment included prednisone and azathioprine for 24 or more months. The majority of patients remained on ursodeoxycholic acid ( UDCA) throughout treatment. Response to therapy was defined by improvement in enzymes, and was rapid for all patients. One patient was able to discontinue treatment with prednisone and azathioprine, while seven have continued on therapy to date. Conclusions: A 'sequential' overlap syndrome of AIH with PBC can occur. Treatment with prednisone and azathioprine may lead to a rapid improvement in aminotransferase levels.