临床病例评析——酷似皮肌炎的副神经节瘤致异位促肾上腺皮质激素综合征一例  

A case of ectopic adrenocorticotropic hormone syndrome caused by paraganglioma mimic dermatomyositis

作　　者：陈达 叶华[1] 周灵丽[2] 高松源[3] 贾晋松[4] 徐燕[5] 刘燕鹰[1] Chen Da;Ye Hua;Zhou Lingli;Gao Songyuan;Jia Jinsong;Xu Yan;Liu Yanying(Department of Rheumatology&Immunology,Peking University People's Hospital,Beijing 100044,China;Department of Endocrinology,Peking University People's Hospital,Beijing 100044,China;Department of Pathology,Peking University People's Hospital,Beijing 100044,China;Department of Hematology,Peking University People's Hospital,Beijing 100044,China;Department of Neurology,Peking University People's Hospital,Beijing 100044,China)

机构地区：[1]北京大学人民医院风湿免疫科,100044 [2]北京大学人民医院内分泌科,100044 [3]北京大学人民医院病理科,100044 [4]北京大学人民医院血液科,100044 [5]北京大学人民医院神经内科,100044

出　　处：《中华风湿病学杂志》2019年第10期662-665,I0002,共5页Chinese Journal of Rheumatology

基　　金：国家重点研发计划(2017YFA0105802);北京大学人民医院研究与发展基金(RDH2017-02)。

摘　　要：目的提高临床对异位促肾上腺皮质激素综合征的认识。方法对1例酷似皮肌炎的43岁女性患者全身多系统受累的诊疗演变过程进行分析讨论。结果患者乏力、水肿、皮肤色素沉着、神经精神异常、高血压、低血钾、高血糖等全身多系统受累,排除其他肿瘤、CTD,诊断为异位促肾上腺皮质激素综合征,病理证实为前纵隔副神经节瘤所致,手术切除肿瘤,患者临床症状消失,激素水平恢复正常。结论副神经节瘤致异位促肾上腺皮质激素综合征临床罕见,非内分泌科医生应提高临床认识。Objective To improve the recognition of ectopic adrenocorticotropic hormone syndrome.Methods The diagnosis and treatment of a 43 years old female patient with multiorgan involvement mimic dermatomyositis was analyzed and discussed.Results The patient presented with fatigue,edema,skin pigmentation,neuropsychiatric abnormalities,hypertension,hypokalemia,hyperglycemia and other systemic involvement,was finally diagnosed with ectopic adrenocorticotropic hormone syndrome caused by paraganglioma of the anterior mediastinum.After surgical removal of the tumor,her clinical symptoms immediately relieved,meanwhile related hormone levels returned to normal.Conclusion Although paraganglioma-induced ectopic adrenocorticotropic hormone syndrome is rare in clinical practice,more attention should be paid to this specific situation.

关 键 词：ACTH综合征 异位 副神经节瘤 

分 类 号：R59[医药卫生—内科学]