Department of Internal Medicine IV, Oncology/Hematology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany.
First Department of Medicine, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany.
Semin Immunopathol. 2022 Jul;44(4):411-427. doi: 10.1007/s00281-022-00937-5. Epub 2022 Apr 29.
B cells are central for the adaptive immune system to mount successful immune responses not only as antibody producers but also as regulators of cellular immunity. These multifaceted features are also reflected in autoimmunity where autoreactive B cells can fuel disease by production of cytotoxic autoantibodies, presentation of autoantigens to autoreactive T cells, and secretion of cytokines and chemokines that either promote detrimental immune activation or impair regulatory T and B cells. The role of B cells and autoantibodies in autoimmune hepatitis (AIH) have been controversially discussed, with typical autoantibodies and hypergammaglobulinemia indicating a key role, while strong HLA class II association suggests T cells as key players. In this review, we summarize current knowledge on B cells in AIH and how different B cell subpopulations may drive AIH progression beyond autoantibodies. We also discuss recent findings of B cell-directed therapies in AIH.
B 细胞是适应性免疫系统的核心，不仅可以作为抗体产生细胞，还可以作为细胞免疫的调节剂，从而成功地引发免疫反应。这些多方面的功能也反映在自身免疫中，自身反应性 B 细胞可以通过产生细胞毒性自身抗体、向自身反应性 T 细胞呈递自身抗原以及分泌细胞因子和趋化因子来促进疾病的发展，这些细胞因子和趋化因子要么促进有害的免疫激活，要么损害调节性 T 和 B 细胞。B 细胞和自身抗体在自身免疫性肝炎（AIH）中的作用一直存在争议，典型的自身抗体和高γ球蛋白血症表明其起关键作用，而强烈的 HLA Ⅱ类基因关联表明 T 细胞是关键因素。在这篇综述中，我们总结了目前关于 AIH 中 B 细胞的知识，以及不同的 B 细胞亚群如何在自身抗体之外驱动 AIH 的进展。我们还讨论了 AIH 中 B 细胞靶向治疗的最新发现。
