Department of Internal Medicine, Faculty of Medical Sciences, Lebanese University, Beirut, Lebanon.
Faculty of Medical Sciences, Lebanese University, Beirut, Lebanon.
Acta Gastroenterol Belg. 2021 Jul-Sep;84(3):477-486. doi: 10.51821/84.3.013.
Benign recurrent intrahepatic cholestasis (BRIC) is a rare genetic disorder that is characterized by episodes of cholestasis followed by complete resolution. The episodic nature of BRIC raises concerns about its possible trigger factors. Indeed, case reports of this orphan disease have associated BRIC to some triggers. In the absence of any reviews, we reviewed BRIC trigger factors and its pathophysiology. The study consisted of a systematic search for case reports using PubMed. Articles describing a clear case of BRIC associated with a trigger were included resulting in 22 articles that describe 35 patients. Infection was responsible for 54.3% of triggered episodes, followed by hormonal, drugs, and miscellaneous causes reporting as 30%, 10%, and 5.7% respectively. Females predominated with 62.9%. The longest episode ranged between 3 months to 2 years with a mean of 32.37 weeks. The mean age of the first episode was 14.28 ranging between 3 months to 48 years. Winter and autumn were the major seasons during which episodes happened. Hence, BRIC is potentially triggered by infection, which is most commonly a viral infection, hormonal disturbances as seen in oral contraceptive pills and pregnancy state, and less commonly by certain drugs and other causes. The appearance of cholestasis during the first two trimesters of pregnancy compared to intrahepatic cholestasis of pregnancy could help to differentiate between the two conditions. The possible mechanism of BRIC induction implicates a role of BSEP and ATP8B1. While estrogen, drugs, and cytokines are known to affect BSEP, less is known about their action on ATP8B1.
良性复发性肝内胆汁淤积症（BRIC）是一种罕见的遗传性疾病，其特征为胆汁淤积发作，随后完全缓解。BRIC 的发作性特征引起了人们对其可能触发因素的关注。事实上，这种孤儿病的病例报告已经将 BRIC 与一些触发因素联系起来。由于缺乏任何综述，我们回顾了 BRIC 的触发因素及其病理生理学。该研究包括使用 PubMed 进行系统的病例报告搜索。纳入了描述与触发因素明确相关的 BRIC 清晰病例的文章，最终纳入了 22 篇描述 35 名患者的文章。感染导致了 54.3%的发作性事件，其次是激素、药物和其他原因，分别占 30%、10%和 5.7%。女性占 62.9%。最长的发作时间在 3 个月至 2 年之间，平均为 32.37 周。首次发作的平均年龄为 14.28 岁，范围为 3 个月至 48 岁。冬季和秋季是发作的主要季节。因此，BRIC 可能由感染引发，最常见的是病毒感染，激素紊乱如口服避孕药和妊娠状态，以及较少见的某些药物和其他原因。与妊娠肝内胆汁淤积症相比，妊娠前两个三个月出现胆汁淤积有助于区分这两种情况。BRIC 诱导的可能机制涉及 BSEP 和 ATP8B1 的作用。虽然雌激素、药物和细胞因子已知会影响 BSEP，但它们对 ATP8B1 的作用知之甚少。
