ImmunoConcEpT, CNRS-UMR 5164 and Université de Bordeaux, Bordeaux, France.
Centre Hospitalier Universitaire de Bordeaux, Service d'Immunologie et Immunogénétique, Bordeaux, France.
Front Immunol. 2020 Jan 30;11:6. doi: 10.3389/fimmu.2020.00006. eCollection 2020.
Common variable immunodeficiency is the most common clinical primary immunodeficiency in adults. Its hallmarks are hypogammaglobulinemia and compromised B-cell differentiation into memory or antibody-secreting cells leading to recurrent infections. This disease is heterogeneous, with some patients harboring multiple complications such as lymphoproliferative disorders, autoimmune manifestations, or granulomatous inflammation. The mechanisms leading to these complications remain elusive despite numerous associations found in the literature. For instance, although described as a B cell intrinsic disease, numerous abnormalities have been reported in other immune cell compartments. Here, we tuned our attention to follicular helper T cells, a CD4 T cell population specialized in B cell help, considering the recent publications showing an involvement of these cells in CVID pathogenesis.
普通变异性免疫缺陷是成人中最常见的临床原发性免疫缺陷。其特征是低丙种球蛋白血症和 B 细胞分化为记忆或抗体分泌细胞受损,导致反复感染。这种疾病具有异质性,有些患者存在多种并发症,如淋巴增生性疾病、自身免疫表现或肉芽肿性炎症。尽管文献中有许多关联,但导致这些并发症的机制仍不清楚。例如,尽管被描述为 B 细胞内在疾病,但在其他免疫细胞群中已报道了许多异常。在这里,我们关注滤泡辅助 T 细胞,这是一种专门辅助 B 细胞的 CD4 T 细胞群,考虑到最近的出版物表明这些细胞参与了 CVID 的发病机制。