第490例--关节痛、停经、言语不利  

The 490th case: arthralgia, amenorrhea, aphasia

作　　者：刘菱珊 赵久良 何泳蓝[2] 宋亦军[3] 曾小峰 Liu Lingshan;Zhao Jiuliang;He Yonglan;Song Yijun;Zeng Xiaofeng(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;Department of Obstetrics&Gynecology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院风湿免疫科,国家皮肤与免疫疾病临床医学研究中心,风湿免疫病学教育部重点实验室,100730 [2]中国医学科学院,北京协和医学院,北京协和医院放射科,100730 [3]中国医学科学院,北京协和医学院,北京协和医院妇产科,100730

出　　处：《中华内科杂志》2021年第12期1189-1192,共4页Chinese Journal of Internal Medicine

摘　　要：患者女,25岁。因关节痛5年,停经16个月,言语不利3个月就诊北京协和医院。患者5年前间断出现双手掌指关节、近端指间关节游走性疼痛,未予特殊诊治。近1年余出现月经不规律,进展至闭经,查雌二醇<5.00 ng/L,孕酮0.058μg/L,卵泡刺激素56.09~62.50 IU/L,促黄体生成素49.32~58.71 IU/L,催乳素198.7μIU/ml,妇科超声检查示无窦状卵泡,考虑卵巢功能早衰,予性激素替代治疗,期间监测仍无排卵。4个月前患者反复出现右上肢麻木无力,3个月前突发言语不利伴右侧肢体无力持续不缓解,查头颅磁共振血管成像(MRA)示急性卒中,停用性激素替代治疗,完善病因筛查,发现抗核抗体、抗双链DNA抗体、抗SSA抗体、抗SSB抗体阳性,抗心磷脂抗体、抗β2糖蛋白Ⅰ抗体阳性,诊断系统性红斑狼疮伴发抗磷脂综合征。结合患者发病初期出现闭经,性激素水平为绝经后水平,此前无性腺毒性药物使用史,考虑患者闭经为系统性红斑狼疮继发自身免疫性卵巢炎可能,予口服泼尼松(60 mg,1次/d,规律减量每周5%)联合吗替麦考酚酯(0.75 g,2次/d)及羟氯喹(0.2 g,2次/d)治疗,4个月后患者月经来潮,此后规律行经至今。通过分析患者的临床资料,提高临床医师对系统性红斑狼疮继发自身免疫性卵巢炎的认识。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-β2GPⅠantibodies.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.

关 键 词：红斑狼疮 系统性 抗磷脂综合征 闭经 自身免疫性卵巢炎 卵巢功能减退 

分 类 号：R593.2[医药卫生—内科学] R711.75[医药卫生—临床医学]