Immune Alterations in Patients with Anti-Interferon-γ Autoantibodies

Autoantibodies against interferon-gamma (IFN-γ) can cause immunodeficiency and are associated with various opportunistic infections. In the present study, we investigated other cellular immune parameters for a better understanding of the immunodeficiency condition in the patients. The numbers of WBC, monocytes and NK cells were increased in patients with anti-IFN-γ autoantibodies (AAbs). Upon TCR activation, T cell proliferation and IL-2 receptor of the patients remained intact. Nonetheless, the Th1 cytokine (IFN-γ and TNF-α) production was up-regulated. The production of Th2 (IL-4) and Th17 (IL-17) cytokines was unchanged. We suggest that, in addition to the presence of anti-IFN-γ autoantibodies, alterations in the cellular immune functions may also contribute to this immunodeficiency.

抗γ干扰素（interferon-gamma, IFN-γ）自身抗体可引发免疫缺陷，并与多种机会性感染密切相关。本研究为深入阐明此类患者的免疫缺陷状态，对其他细胞免疫参数展开了检测分析。抗IFN-γ自身抗体（AAbs）阳性患者的白细胞（white blood cell, WBC）、单核细胞及自然杀伤（natural killer cell, NK）细胞计数均显著升高。经T细胞受体（T cell receptor, TCR）激活后，患者的T细胞增殖能力与白细胞介素-2（interleukin-2, IL-2）受体表达均保持完整。尽管如此，辅助性T细胞1型（T helper cell 1, Th1）分泌的细胞因子（IFN-γ及肿瘤坏死因子-α（tumor necrosis factor-α, TNF-α））的产生出现上调；而辅助性T细胞2型（T helper cell 2, Th2，分泌白细胞介素-4（interleukin-4, IL-4））与辅助性T细胞17型（T helper cell 17, Th17，分泌白细胞介素-17（interleukin-17, IL-17））的细胞因子产生则无明显变化。我们认为，除抗IFN-γ自身抗体的存在外，细胞免疫功能的异常或许也参与了此类免疫缺陷的发生发展。