IFN-γ抗体导致的免疫缺陷综合征
胸科之窗
这不仅仅是一个公众号....
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.