胸腺瘤伴有免疫缺陷、合并弥漫性泛细支气管炎和潜伏性自身免疫性糖尿病的成人--病例报告和系统综述

IF 4.7 Q2 IMMUNOLOGY Journal of Translational Autoimmunity Pub Date : 2023-12-24 DOI:10.1016/j.jtauto.2023.100230
Yijiao Xu , Lumin Wang , Zhisheng Chen , Qingwei Zhang , Yun Shen , Yanrong Ye , Jiaxin Liu , Huijun Zhang
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引用次数: 0

摘要

胸腺瘤伴免疫缺陷综合征(Good's Syndrome,GS)是胸腺瘤与免疫缺陷之间的一种罕见关联,60 多年前首次被描述。GS患者通常表现为胸腺瘤、外周血中B细胞减少或缺失、低丙种球蛋白血症和细胞介导的免疫缺陷。我们报告了一例 67 岁女性患者的病例,她在出现进行性、严重、难治性肺部感染和弥漫性泛细支气管炎(DPB)后被诊断为 GS。她还患有以抗谷氨酸脱羧酶抗体阳性为特征的糖尿病,因此被诊断为成人潜伏性自身免疫性糖尿病(LADA)。对现有文献的深入研究表明,胸腺瘤术后多次发生机会性感染或自身免疫性疾病后,通常会确诊为 GS。由于免疫缺陷,GS 患者经常会长期反复感染,有些患者会因严重感染而死亡。定期输注免疫球蛋白可有效治疗 GS。
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Thymoma with immunodeficiency, combined diffuse panbronchiolitis, and latent autoimmune diabetes in adults- case report and systematic review

Thymoma with Immunodeficiency (Good's Syndrome, GS) is a rare association between thymoma and immunodeficiency, first described over 60 years ago. Patients with GS typically present with thymomas, reduced or absent B cells in the peripheral blood, hypogammaglobulinemia, and defects in cell-mediated immunity. We report the case of a 67-year-old woman diagnosed with GS following the development of a progressive, severe, refractory pulmonary infection and diffuse panbronchiolitis (DPB). She also had diabetes, characterized by anti-glutamic acid decarboxylase antibody positivity, leading to a diagnosis of latent autoimmune diabetes in adults (LADA). A thorough review of existing literature revealed that GS is often confirmed after multiple episodes of opportunistic infections or autoimmune diseases post-thymoma surgery. Due to their immunodeficiency, GS patients frequently suffer from recurrent infections over extended periods, and some succumb to severe infections. Regular immunoglobulin infusions may be effective in treating GS.

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来源期刊
Journal of Translational Autoimmunity
Journal of Translational Autoimmunity Medicine-Immunology and Allergy
CiteScore
7.80
自引率
2.60%
发文量
33
审稿时长
55 days
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