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米非司酮致库欣综合征患者肝损伤 1 例报告并文献复习

Mifepristone induced liver injury in a patient with Cushing syndrome: a case report and review of the literature.

机构信息

Hoag Digestive Health Institute, Hoag Memorial Hospital Presbyterian, One Hoag Drive, Newport Beach, CA, 92663, USA.

Department of Pathology, Hoag Memorial Hospital Presbyterian, Newport Beach, CA, USA.

出版信息

J Med Case Rep. 2023 Feb 3;17(1):33. doi: 10.1186/s13256-022-03696-x.

Abstract

BACKGROUND

Mifepristone, also known as RU-486, is an anti-progestational steroid with similar chemical structure to anabolic steroids. Given as a single dose in conjunction with misoprostol, mifepristone is used to induce medical abortion. Mifepristone administered chronically at a higher dose is also approved for the management of hypercortisolism. There have been only 2 reported cases of mifepristone associated liver injury, in both cases, in the setting of Cushing syndrome. We report a third patient with Cushing syndrome with mifepristone induced liver injury with unique histological findings that provide insight to the pathophysiology of liver injury in mifepristone and anabolic steroids.

CASE PRESENTATION

Patient is a 63-year-old Caucasian female Cushing disease with no prior history of liver disease. She was started on mifepristone and semaglutide. Ninety days after initiating mifepristone, she developed deep jaundice, severe pruritus, fatigue, and nausea. Liver tests revealed a mixed hepatocellular/cholestatic pattern. Viral and autoimmune serologies were negative and there was no biliary dilatation on imaging. Liver biopsy showed severe cholestasis but no bile duct injury. Focal endothelialitis was present within a central venule. Cholestatic symptoms persisted for one month after presentation before slowly subsiding. Four months after stopping mifepristone, the patient's symptoms completely resolved, and liver tests became normal. Compilation of Roussell Uclaf Causality Assessment Method score indicated probable causality.

CONCLUSIONS

Mifepristone shares a similar chemical structure as synthetic anabolic/androgenic steroids and there are many similarities in the clinical presentation of liver injury. This case and the 2 other reported cases share similar clinical characteristics. The observation of endothelialitis in our patient may provide a mechanistic link between mifepristone, or anabolic steroids in general, and the development of vascular complications such as peliosis.

摘要

背景

米非司酮,也称为 RU-486,是一种具有类似化学结构的抗孕激素甾体,与合成代谢类固醇相似。与米索前列醇联合使用,单次给药用于诱导药物流产。米非司酮以更高剂量长期给药也被批准用于治疗皮质醇增多症。仅报道了 2 例与米非司酮相关的肝损伤病例,在这两种情况下,都是在库欣综合征的背景下。我们报告了第 3 例库欣综合征患者,使用米非司酮引起肝损伤,其独特的组织学发现为米非司酮和合成代谢类固醇引起的肝损伤的病理生理学提供了见解。

病例介绍

患者为 63 岁白人女性,库欣病,无既往肝脏疾病史。她开始服用米非司酮和司美格鲁肽。在开始使用米非司酮 90 天后,她出现深度黄疸、严重瘙痒、疲劳和恶心。肝功能检查显示混合性肝细胞/胆汁淤积模式。病毒和自身免疫血清学检查均为阴性,影像学检查未见胆管扩张。肝活检显示严重胆汁淤积,但无胆管损伤。中央静脉内存在局灶性内皮炎。在就诊后一个月,胆汁淤积症状持续缓慢消退。在停止使用米非司酮 4 个月后,患者的症状完全缓解,肝功能检查恢复正常。Roussel Uclaf Causality Assessment Method 评分表明可能与因果关系有关。

结论

米非司酮的化学结构与合成代谢/雄激素类固醇相似,肝损伤的临床表现也有许多相似之处。本病例和另外 2 例报道的病例具有相似的临床特征。我们的患者观察到内皮炎,这可能为米非司酮或一般合成代谢类固醇与血管并发症(如肝血窦扩张)的发展之间提供了一种机制联系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/761c/9896794/b5f712397206/13256_2022_3696_Fig1_HTML.jpg

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