Fevang Børre, Yndestad Arne, Damås Jan K, Bjerkeli Vigdis, Ueland Thor, Holm Are M, Beiske Klaus, Aukrust Pål, Frøland Stig S
Research Institute for Internal Medicine, Rikshospitalet, University of Oslo, N-0027 Oslo, Norway.
Clin Immunol. 2009 Feb;130(2):151-61. doi: 10.1016/j.clim.2008.09.002. Epub 2008 Oct 19.
Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by defective immunoglobulin production and high frequency of bacterial infections, autoimmunity and manifestations of chronic inflammation. The chemokine Fractalkine (CX3CL1) and its receptor CX3CR1 is suggested to play an important role in the pathogenesis of several inflammatory disorders. We hypothesized that enhanced CX3CL1/CX3CR1 interaction could be involved in the chronic inflammation characterising subgroups of CVID. CVID patients were characterized by raised plasma levels of CX3CLl and enhanced expression of its corresponding receptor CX3CR1 on CD4(+) and CD8(+) T cells, including both CD45RA(+) and CD45RA(-) subsets. CX3CR1 expression was particularly enhanced in patients characterized by chronic inflammation in vivo. The high expression of the receptor in CVID patients was accompanied by enhanced chemotactic, adhesive, and other inflammatory cell responses to stimulation with CX3CL1. Our findings suggest that increased CX3CL1/CX3CR1 interaction could contribute to the inflammatory phenotype seen in subgroups of CVID patients.
普通可变免疫缺陷（CVID）是一种异质性综合征，其特征为免疫球蛋白产生缺陷、细菌感染频率高、自身免疫以及慢性炎症表现。趋化因子Fractalkine（CX3CL1）及其受体CX3CR1被认为在几种炎症性疾病的发病机制中起重要作用。我们推测，增强的CX3CL1/CX3CR1相互作用可能与CVID亚组的慢性炎症有关。CVID患者的特征是血浆中CX3CL1水平升高，其相应受体CX3CR1在CD4(+)和CD8(+) T细胞（包括CD45RA(+)和CD45RA(-)亚群）上的表达增强。CX3CR1表达在体内以慢性炎症为特征的患者中尤其增强。CVID患者中该受体的高表达伴随着对CX3CL1刺激的趋化、黏附及其他炎症细胞反应增强。我们的研究结果表明，CX3CL1/CX3CR1相互作用增强可能导致CVID患者亚组中出现的炎症表型。
