第 11 次专题讨论会

Janet Wong
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引用次数: 0

摘要

自身免疫性疾病是一种免疫功能紊乱的疾病,会导致不适当的适应性免疫过度激活,进而损害宿主的各种组织。在风湿病诊所就诊的自身免疫性疾病(类风湿性关节炎、系统性红斑狼疮或免疫介导的肌炎)通常采用免疫抑制治疗,这几乎无一例外地与感染易感性增加有关。不过,免疫抑制有时也可反过来治疗易感染性。越来越多的人认识到,有一类新出现的自身免疫性疾病以宿主自身的免疫系统(或相关细胞因子)为靶点,在表型上模仿各种免疫缺陷综合征,即所谓的 "PID 表型"。在我们当地,越来越多的人认识到一种特殊的 PID 表型,即孟德尔分枝杆菌病易感性(MSMD)。这种 "获得性 MSMD "是由于抗 IFN[公式:见正文]的自身抗体导致对分枝杆菌和其他 "IFN-[公式:见正文]依赖性 "感染的易感性增加。与其他自身免疫性疾病一样,适当和有针对性的免疫抑制可以减轻抗IFN-[式:见正文]抗体的影响,并有助于相关感染的治疗。在本讲座中,我们将讨论本地获得性 MSMD 的诊断和管理经验。我们还将分享新型诊断和功能测试的结果,以及获得性 MSMD 患者接受 B 细胞耗竭疗法的疗效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Symposium 11
Autoimmune diseases are conditions of immune dysfunction, leading to inappropriate hyperactivation of the adaptive immunity and subsequent damage to various tissues of the host. Autoimmune conditions presenting in the rheumatology clinics (rheumatoid arthritis, systemic lupus erythematosus or immune mediated myositis) are often treated by immunosuppression, which are almost invariably associated with increased susceptibility to infections. However, immunosuppression can sometimes be used to conversely treat susceptibility to infections. An emerging group of autoimmune diseases which target the host’s own immune system (or associated cytokines) are becoming increasing recognized which phenotypically mimics various immunodeficiency syndromes – known as “phenocopies of PID”. One particular phenocopy of a PID known as Mendelian Susceptibility to Mycobacterial Disease (MSMD) has been increasingly recognized in our locality. This “acquired MSMD” is due to autoantibodies against anti-IFN[Formula: see text], leading to increased susceptibility to mycobacterial and other “IFN-[Formula: see text] dependent” infections. Alike other autoimmune diseases, appropriate and targeted immunosuppression can alleviate the effect of anti-IFN-[Formula: see text] antibodies and aid treatment of associated infections. During this talk, our experience in the diagnosis and management toward acquired MSMD in our locality will be discussed. We will also share findings from our novel diagnostic and functional tests, as well as outcomes of acquired MSMD patients treated with B-cell depleting therapies.
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