Chiunti-Andrade Práxedes, Gallardo-Hernández Carlos Arturo, González-Herrera Sandra Luz
Universidad Veracruzana, Unidad Académica de Ciencias de la Salud, Facultad de Bioanálisis. Xalapa, Veracruz, México.
Rev Med Inst Mex Seguro Soc. 2023 Mar 1;61(2):189-195.
The autoimmune diseases include many in which the immune system is directed against the host, leading to life-threatening destruction of organs. The origin of autoimmune disorders can be multifactorial and, there are no specific therapy for these diseases. Primary immunodeficiencies are a group of immune disorders that affect different components of the innate and adaptive responses. Interestingly, patients with primary immunodeficiencies have an increased susceptibility to infectious diseases and non-infectious complications including allergies, malignancies, and autoimmune diseases. The molecular mechanism for development of autoimmunity in immunodeficiencies is unclear. The study of the complex immune regulatory and signaling mechanisms is revealing the relationships between primary immunodeficiency syndromes and autoimmune diseases. Newly, it has been demonstrated that a deficient maturation of immune cells; the deficiency of proteins important for T and B lymphocyte function and impaired signally pathways that include key molecules in regulation and activation of immune cells are associated with the development of autoimmunity in patients with primary immunodeficiencies. The aim of the present work is to review the evidence available to date regarding the cellular and molecular mechanisms involved in the development of autoimmunity in patients with primary immunodeficiencies.
自身免疫性疾病包括许多免疫系统攻击宿主从而导致危及生命的器官破坏的疾病。自身免疫性疾病的起源可能是多因素的，并且这些疾病没有特异性治疗方法。原发性免疫缺陷是一组影响先天性和适应性免疫反应不同组成部分的免疫疾病。有趣的是，原发性免疫缺陷患者对包括过敏、恶性肿瘤和自身免疫性疾病在内的传染病和非感染性并发症的易感性增加。免疫缺陷中自身免疫性疾病发生的分子机制尚不清楚。对复杂免疫调节和信号传导机制的研究正在揭示原发性免疫缺陷综合征与自身免疫性疾病之间的关系。最近，已经证明免疫细胞成熟缺陷、对T和B淋巴细胞功能重要的蛋白质缺乏以及包括免疫细胞调节和激活中的关键分子在内的信号通路受损与原发性免疫缺陷患者自身免疫性疾病的发生有关。本研究的目的是综述迄今为止关于原发性免疫缺陷患者自身免疫性疾病发生所涉及的细胞和分子机制的现有证据。
