Abstract：
Whereas autoimmune diseases are mediated primarily by T and B cells, auto-inflammatory syndromes (AIFS) involve natural killer cells, macrophages, mast cells, dendritic cells, different granulocyte subsets and complement components. In contrast to autoimmune diseases, the immune response of patients with AIFS is not associated with a breakdown of immune tolerance to self-antigens. Focusing on B lymphocyte subsets, this article offers a fresh perspective on the multiple cross-talks between both branches of innate and adaptive immunity in mounting coordinated signals that lead to AIFS. By virtue of their potential to play a role in adaptive immunity and to exert innate-like functions, B cells can be involved in both promoting inflammation and mitigating auto-inflammation in disorders that include mevalonate kinase deficiency syndrome, Kawasaki syndrome, inflammatory bone disorders, Schnitzler syndrome, Neuro-Behçet\'s disease, and neuromyelitis optica spectrum disorder. Since there is a significant overlap between the pathogenic trajectories that culminate in autoimmune diseases, or AIFS, a more detailed understanding of their respective roles in the development of inflammation could lead to designing novel therapeutic avenues.
摘要：
而自身免疫性疾病主要由T和B细胞介导，自身炎症综合征（AIFS）涉及自然杀伤细胞，巨噬细胞，肥大细胞,树突状细胞,不同的粒细胞亚群和补体成分。与自身免疫性疾病相反，AIFS患者的免疫应答与自身抗原免疫耐受的破坏无关.关注B淋巴细胞亚群，这篇文章提供了一个全新的视角,即先天性免疫和适应性免疫两个分支之间在安装导致AIFS的协调信号时的多重交叉对话.凭借其在适应性免疫中发挥作用和发挥先天样功能的潜力，在包括甲羟戊酸激酶缺乏综合征在内的疾病中，B细胞可参与促进炎症和减轻自身炎症。川崎综合征,炎症性骨病，Schnitzler综合征,神经-Behçet病,和视神经脊髓炎谱系障碍。由于最终导致自身免疫性疾病的致病轨迹之间存在显着的重叠，或AIFS，更详细地了解它们在炎症发展中的作用可能会导致设计新的治疗途径。
