Xu Yijiao, Wang Lumin, Chen Zhisheng, Zhang Qingwei, Shen Yun, Ye Yanrong, Liu Jiaxin, Zhang Huijun
Zhongshan Hospital (Xiamen), Fudan University, 361015, China.
Zhongshan Hospital, Fudan University, 200032, China.
J Transl Autoimmun. 2023 Dec 24;8:100230. doi: 10.1016/j.jtauto.2023.100230. eCollection 2024 Jun.
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.
胸腺瘤伴免疫缺陷（古德综合征，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B细胞减少或缺失、低丙种球蛋白血症以及细胞介导免疫缺陷。我们报告了一例67岁女性病例，该患者在发生进行性、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。她还患有糖尿病，其特征为抗谷氨酸脱羧酶抗体阳性，导致诊断为成人隐匿性自身免疫性糖尿病（LADA）。对现有文献的全面回顾显示，GS通常在胸腺瘤手术后多次发生机会性感染或自身免疫性疾病后得到确诊。由于免疫缺陷，GS患者经常在很长一段时间内反复感染，一些患者死于严重感染。定期输注免疫球蛋白可能对治疗GS有效。
