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[第490例:关节痛、闭经、失语]

[The 490th case: arthralgia, amenorrhea, aphasia].

作者信息

Liu L S, Zhao J L, He Y L, Song Y J, Zeng X F

机构信息

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China.

Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

出版信息

Zhonghua Nei Ke Za Zhi. 2021 Dec 1;60(12):1189-1192. doi: 10.3760/cma.j.cn112138-20210425-00308.

Abstract

A 25-year-old woman was admitted to Peking Union Medical Hospital presented with arthralgia for 5 years, amenorrhea for 16 months, and speech disorder for 3 months. This patient has been afflicted by intermittent pain in metacarpophalangeal and proximal interphalangeal joints of both hands for 5 years. Her menstruation has been irregular 1 year ago and rapidly progressed to amenorrhea. Laboratory tests revealed postmenopausal sex hormones levels (estradiol<5 ng/L, follicle-stimulating hormone 62.5 IU/L, luteinizing hormone 58.71 IU/L) and no antral follicles were seen in gynecologic ultrasound. She was diagnosed with premature ovarian failure and treated with hormone replacement therapy, still with no ovulation. Numbness and weakness of right arm has recurrently occurred to her 4 months ago, and persistent weakness of right limbs combined with motor speech disorder occurred 1 month later. Magnetic resonance angiography was suggestive of ischemic stroke. Hormone replacement therapy was discontinued. Comprehensive laboratory tests revealed positive anti-dsDNA, anti-SSA/SSB, anticardiolipin and anti-βGPⅠ antibodies. Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS) was diagnosed. Since no drug with gonadal toxicity had been applied to the patient before, her amenorrhea was considered to be due to autoimmune oophoritis secondary to SLE. After treated with high-dose glucocorticoid, mycophenolate mofetil and hydroxychloroquine for 4 months, her menstruation recurred and regularly occurred till now. In some cases, amenorrhea in SLE patient might be resulted from autoimmune oophoritis associated with lupus flare, instead of use of drug with gonadal toxicity.

摘要

一名25岁女性因关节痛5年、闭经16个月、言语障碍3个月入住北京协和医院。该患者双手掌指关节和近端指间关节间歇性疼痛5年。1年前月经开始不规律,并迅速发展为闭经。实验室检查显示绝经后性激素水平(雌二醇<5 ng/L,促卵泡生成素62.5 IU/L,促黄体生成素58.71 IU/L),妇科超声未见窦卵泡。她被诊断为卵巢早衰并接受激素替代治疗,但仍无排卵。4个月前她右臂反复出现麻木和无力,1个月后出现右下肢持续无力并伴有运动性言语障碍。磁共振血管造影提示缺血性卒中。停用激素替代治疗。综合实验室检查显示抗双链DNA、抗SSA/SSB、抗心磷脂和抗β2糖蛋白Ⅰ抗体阳性。诊断为系统性红斑狼疮(SLE)、抗磷脂综合征(APS)。由于患者此前未使用过具有性腺毒性的药物,其闭经被认为是继发于SLE的自身免疫性卵巢炎所致。经大剂量糖皮质激素、霉酚酸酯和羟氯喹治疗4个月后,月经恢复且至今规律来潮。在某些情况下,SLE患者的闭经可能是由与狼疮活动相关的自身免疫性卵巢炎引起,而非使用具有性腺毒性的药物所致。

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