Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium; Laboratory for Inborn Errors of Immunity, Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium.
Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium; Laboratory for Inborn Errors of Immunity, Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium.
Curr Opin Immunol. 2021 Oct;72:331-339. doi: 10.1016/j.coi.2021.08.004. Epub 2021 Sep 17.
The binary view of inborn errors of immunity classified as either autoinflammatory conditions or primary immunodeficiency in the strict sense, that is, increased susceptibility to infection is challenged by the description of recent inborn errors of immunity (IEI) triggers leading to activation and disruption of cell death pathways, play a major part in the pathophysiology of infection and autoinflammation. In addition, molecules with a double role in the extracellular versus intracellular milieu add to the complexity. In all, in-depth study of human inborn errors of immunity will continue to instruct us on fundamental immunology and lead to novel therapeutic targets and approaches that can be used in other monogenic and polygenic/complex immune disorders.
先天性免疫错误的二分法，要么被归类为自身炎症性疾病，要么被归类为严格意义上的原发性免疫缺陷，即对感染的易感性增加，这一观点受到了挑战，因为最近描述的先天性免疫错误（IEI）触发因素导致细胞死亡途径的激活和破坏，在感染和自身炎症的病理生理学中起着重要作用。此外，在细胞外环境和细胞内环境中具有双重作用的分子增加了复杂性。总之，对人类先天性免疫错误的深入研究将继续为我们提供基础免疫学方面的知识，并为其他单基因和多基因/复杂免疫疾病提供新的治疗靶点和方法。
