Liang Xiao-Na, Bin Yan-Fei, Lai Guan-Ting, Li Ying-Hua, Zhang Jian-Quan, Zhong Xiao-Ning, Bai Jing, Li Mei-Hua, Deng Jing-Min, He Zhi-Yi
Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, P.R. China.
Medicine (Baltimore). 2020 Sep 4;99(36):e21738. doi: 10.1097/MD.0000000000021738.
Anti-interferon-gamma (anti-IFN-γ) autoantibody increases susceptibility to lower-virulence pathogens and causes immunodeficiency syndrome in HIV-negative patients.
A 69-year-old Chinese man presented with a 2-month history of pruritic skin lesions on his forearms, trunk, and legs. He was diagnosed with 5 opportunistic infections without conventional immunosuppression-associated factors in past. The most conspicuous characteristics were recurrent pulmonary infection, persistent immunoglobulin E elevation and eosinophilia during the whole disease course.
Enzyme-linked immunosorbent assay showed anti-IFN-γ autoantibody positive. The final diagnosis for the patient was adult-onset immunodeficiency due to anti-IFN-γ autoantibody, non-tuberculous mycobacterial (NTM) infection and reactive dermatosis.
The patient underwent long-term anti-NTM and corticosteroid maintenance treatment.
The patient was followed for 2 years during which opportunistic infection no longer happened, the immunoglobulin E level and eosinophil count reduced, the autoantibody levels remained largely steady and lung lesions absorbed.
Clinicians should be vigilant for NTM infection in patients with anti-IFN-γ autoantibodies, even when culture results are negative. Long-term anti-non-tuberculous mycobacteria and glucocorticoid regimens were effective.
抗干扰素-γ(抗IFN-γ)自身抗体增加了对低毒力病原体的易感性,并在HIV阴性患者中导致免疫缺陷综合征。
一名69岁中国男性,前臂、躯干和腿部出现瘙痒性皮肤病变2个月。他曾被诊断出5次机会性感染,既往无传统免疫抑制相关因素。最显著的特点是在整个病程中反复出现肺部感染、持续性免疫球蛋白E升高和嗜酸性粒细胞增多。
酶联免疫吸附试验显示抗IFN-γ自身抗体阳性。该患者最终诊断为成人抗IFN-γ自身抗体所致免疫缺陷、非结核分枝杆菌(NTM)感染和反应性皮肤病。
患者接受了长期抗NTM和糖皮质激素维持治疗。
对患者进行了2年随访,期间未再发生机会性感染,免疫球蛋白E水平和嗜酸性粒细胞计数降低,自身抗体水平基本保持稳定,肺部病变吸收。
临床医生应警惕抗IFN-γ自身抗体患者的NTM感染,即使培养结果为阴性。长期抗非结核分枝杆菌和糖皮质激素治疗方案有效。