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Autoimmune polyglandular syndrome type 2 and autoimmune hepatitis with thymoma-associated myasthenia gravis: case report.
BMC Endocrine Disorders ( IF 2.8 ) Pub Date : 2020-04-07 , DOI: 10.1186/s12902-020-0498-5
Hidefumi Inaba 1 , Hiroyuki Ariyasu 1 , Hiroshi Iwakura 1 , Chiaki Kurimoto 1 , Yoko Ueda 1 , Shinsuke Uraki 1 , Ken Takeshima 1 , Yasushi Furukawa 1 , Shuhei Morita 1 , Yoshiaki Nakayama 2 , Takuya Ohashi 3 , Hidefumi Ito 2 , Yoshiharu Nishimura 3 , Takashi Akamizu 1
Affiliation  

BACKGROUND Autoimmune polyglandular syndrome type 2 (APS-2) is a rare and complex clinical entity, and little is known about its etiology and progression. CASE PRESENTATION A 52-year-old woman with autoimmune hepatitis (AIH) and bronchial asthma was diagnosed with APS-2; autoimmune Addison's disease (AD), and Hashimoto's thyroiditis (HT), and she underwent prednisolone (PSL) treatment. Five months later, she presented ptosis and was diagnosed with thymoma-associated myasthenia gravis (MG). Thymectomy and PSL treatment with immuno-suppressants appeared to ameliorate MG, AD, AIH, HT, and bronchial asthma. HLA typing analysis revealed that the patient had susceptible HLA alleles to MG, AIH, and HT in a Japanese population. CONCLUSIONS This case suggests common endocrinological and autoimmune aspects of APS-2 and AIH with thymoma-associated MG, which are considered to be extremely rare complications.

中文翻译:

2型自身免疫性多腺综合征和伴胸腺瘤相关的重症肌无力的自身免疫性肝炎:病例报告。

背景技术2型自身免疫性多腺综合征(APS-2)是一种罕见而复杂的临床实体,对其病因和进展知之甚少。病例介绍一名患有自身免疫性肝炎(AIH)和支气管哮喘的52岁女性被诊断出患有APS-2;自身免疫性艾迪生氏病(AD)和桥本甲状腺炎(HT),她接受了泼尼松龙(PSL)治疗。5个月后,她出现下垂,并被诊断患有胸腺瘤相关的重症肌无力(MG)。用免疫抑制剂进行胸腺切除术和PSL治疗似乎可以改善MG,AD,AIH,HT和支气管哮喘。HLA分型分析显示,该患者在日本人群中对MG,AIH和HT易感的HLA等位基因。
更新日期:2020-04-22
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