Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands.
Eur J Neurol. 2015 Aug;22(8):1151-61. doi: 10.1111/ene.12758. Epub 2015 May 29.
At least 13 different disease entities affecting the central nervous system, peripheral nervous system and connective tissue of the skin or kidneys are associated with immunoglobulin G4 (IgG4) immune reactivity. IgG4 has always been considered a benign, non-inflammatory subclass of IgG, in contrast to the well-known complement-activating pro-inflammatory IgG1 subclass. A comprehensive review of these IgG4 autoimmune disorders reveals striking similarities in epitope binding and human leukocyte antigen (HLA) associations. Mechanical interference of extracellular ligand-receptor interactions by the associated IgG4 antibodies seems to be the common/converging disease mechanism in these disorders.
至少有 13 种不同的疾病实体影响中枢神经系统、周围神经系统以及皮肤或肾脏的结缔组织，这些疾病与免疫球蛋白 G4（IgG4）免疫反应有关。与众所周知的补体激活的促炎 IgG1 亚类相反，IgG4 一直被认为是良性的、非炎症性的 IgG 亚类。对这些 IgG4 自身免疫性疾病的全面回顾揭示了表位结合和人类白细胞抗原（HLA）相关性方面的显著相似性。相关 IgG4 抗体对细胞外配体-受体相互作用的机械干扰似乎是这些疾病的共同/趋同的疾病机制。
