IFN-γ抗体导致的免疫缺陷综合征

胸科之窗

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深圳市第三人民医院  

张培泽

01

IFN-γ抗体导致的免疫缺陷综合征

• 与抗IFN-γ抗体相关的表现为播散性非结核分枝感染、机会性感染的非HIV免疫缺陷综合征。

• 主要见于东南亚地区

• 国内广西较早进行报道

• 成人多见，陆续有儿童青少年病例报道

02

临床特点

• 多数成人发病

• 播散性NTM感染

• 播散真菌感染

• 沙门氏菌反复感染

• 合并反应性皮肤病

• 部分有sweet综合征

• 无明确细胞免疫、体液免疫缺陷依据

• 血清IFN-γ抗体IgG高滴度

• 有文献报道血清IFN-γ抗体在病情控制后下降，可能作为疾病转归的生物标志物

A.新型隐球菌感染  B.脓肿分枝杆菌播散性感染  C.  Sweet皮炎

03

发病机制

04

治疗

• 病原学治疗

• 针对IFN-γ抗体导致的免疫缺陷的治疗：

丙种球蛋白

激素

 Rituximab 

CTX

 IFN-γ

……

05

病例分享

• 女性，66岁，反复发热伴淋巴结肿大6月；

• 查体：颈部、腋窝、腹股沟多部位淋巴结肿大，质地偏硬，部分溃破流脓

• 外院颈部淋巴结活检：肉芽肿性炎，NGS:哥伦比亚分枝杆菌

• 肺活检：肉芽肿性炎；

• 肺泡灌洗液：一条抗酸杆菌，NGS:哥伦比亚分枝杆菌

06

小 结

• 认识IFN-γ抗体导致的免疫缺陷综合征的临床特点：播散性特殊病原体感染

• 诊断方法：检测血清IFN-γ抗体

• 治疗：针对病原体治疗及免疫治疗

07

参考文献

1.Browne SK, Burbelo PD, Chetchotisakd P, et al. Adult-onset immunodeficiency in Thailand and Taiwan. N Engl J Med. 2012;367(8):725-734.

2.Poulin S, Corbeil C, Nguyen M, et al. Fatal Mycobacterium colombiense/cytomegalovirus coinfection associated with acquired immunodeficiency due to autoantibodies against interferon gamma: a case report. BMC Infect Dis. 2013;13:24.

3.Tang BS, Chan JF, Chen M, et al. Disseminated penicilliosis, recurrent bacteremic nontyphoidal salmonellosis, and burkholderiosis associated with acquired immunodeficiency due to autoantibody against gamma interferon. Clin Vaccine Immunol. 2010;17(7):1132-1138.

4.Yoshizawa K, Aoki A, Shima K, et al. Serum Anti-interferon-gamma Autoantibody Titer as a Potential Biomarker of Disseminated Non-tuberculous Mycobacterial Infection. J Clin Immunol. 2020;40(2):399-405.

5.Aoki A, Sakagami T, Yoshizawa K, et al. Clinical Significance of Interferon-γ Neutralizing Autoantibodies Against Disseminated Nontuberculous Mycobacterial Disease. Clin Infect Dis. 2018;66(8):1239-1245.

6.Harada M, Furuhashi K, Karayama M, et al. Subcutaneous injection of interferon gamma therapy could be useful for anti-IFN-gamma autoantibody associated disseminated nontuberculous mycobacterial infection. J Infect Chemother. 2020.

7.Czaja CA, Merkel PA, Chan ED, et al. Rituximab as successful adjunct treatment in a patient with disseminated nontuberculous mycobacterial infection due to acquired anti-interferon-gamma autoantibody. Clin Infect Dis. 2014;58(6):e115-118.