Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, France.
Cells. 2022 Apr 4;11(7):1218. doi: 10.3390/cells11071218.
Myasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IFN-I) signature is clearly detected in the thymus. The origin of this chronic IFN-I expression in the thymus is not yet defined. IFN-I subtypes are normally produced in response to viral infection. However, genetic diseases called interferonopathies are associated with an aberrant chronic production of IFN-I defined as sterile inflammation. Some systemic autoimmune diseases also share common features with interferonopathies. This review aims to analyze the pathogenic role of IFN-I in these diseases as compared to AChR-MG in order to determine if AChR-MG could be an acquired interferonopathy.
重症肌无力（MG）是一种罕见的自身免疫性疾病，由针对神经肌肉接头成分的抗体介导，特别是乙酰胆碱受体（AChR）。胸腺在 AChR-MG 患者中起着主要作用。在早发性 AChR-MG 和胸腺瘤相关 MG 中，明显在胸腺中检测到干扰素 I 型（IFN-I）特征。这种慢性 IFN-I 在胸腺中的表达的起源尚未确定。IFN-I 亚型通常是在病毒感染时产生的。然而，称为干扰素病的遗传疾病与 IFN-I 的异常慢性产生有关，这种产生被定义为无菌性炎症。一些系统性自身免疫性疾病也与干扰素病有共同的特征。本综述旨在分析 IFN-I 在这些疾病中的致病作用，与 AChR-MG 进行比较，以确定 AChR-MG 是否可能是一种获得性干扰素病。
