Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Lupus. 2011 Jul;20(8):845-54. doi: 10.1177/0961203311398513. Epub 2011 May 16.
Systemic lupus erythematosus (SLE) is a prototype of systemic autoimmune disease in which cytokines such as B lymphocyte chemoattractant (BLC) or CXC motif ligand 13 (CXCL13) play an important role as major regulators of B1 and B2 cell trafficking for activation of autoreactive T helper cells. CXCL13 can induce trafficking of the CXCR5+ T lymphocyte subset designated as follicular helper T lymphocytes which are specifically involved in autoantibody production during the development of lupus. Here, we ask whether serum levels of CXCL13 correlate with disease activity and severity and renal involvement in children with SLE.
Serum samples from 40 children with SLE and 32 healthy controls were analyzed by ELISA for the concentrations of CXCL13.
Median (interquartile range (IQR) serum CXCL13 concentrations (pg/ml) were increasingly higher across the following groups: healthy controls (71.6 (66.6-81.8), SLE patients with inactive disease (140.8 (99.7-198.8), p = 0.0005 versus controls) and active disease (293.0 (105.5-489.8), p = 0.0001 versus controls) (inactive versus active; p < 0.0001). Concentrations of circulating CXCL13 correlated with SLEDAI (r = 0.499, p = 0.029) and double-stranded DNA titers (r = 0.71, p < 0.001). Moreover, median CXCL13 concentrations were higher in patients with renal involvement (270.6 (150.4-430.7) compared with those without renal involvement (120.6 (70.5-208.9). According to WHO pathological classification of lupus nephritis, median CXCL13 concentrations were higher in children with class III, IV and V nephritis compared with those with class I and II nephritis (333.9 (169.4-491.5) versus (180.4 (107.9-209.7).
Our data indicate that an increased level of CXCL13 is a feature of SLE that correlates with disease activity and severity. CXCL13 expression in lupus nephritis represents a new pathogenetic mechanism of diagnostic and prognostic significance. The pharmacological regulation of CXCL13 and its receptor, CXCR5, expression may be a useful tool in the therapy of lupus nephritis.
系统性红斑狼疮（SLE）是一种系统性自身免疫性疾病的典型范例，其中细胞因子如 B 淋巴细胞趋化因子（BLC）或 CXC 基序配体 13（CXCL13）作为 B1 和 B2 细胞向激活的自身反应性 T 辅助细胞迁移的主要调节剂发挥着重要作用。CXCL13 可诱导 CXCR5+T 淋巴细胞亚群的迁移，该亚群被指定为滤泡辅助 T 淋巴细胞，其在狼疮的发展过程中特异性地参与自身抗体的产生。在这里，我们询问血清 CXCL13 水平是否与儿童 SLE 的疾病活动和严重程度以及肾脏受累相关。
通过 ELISA 分析 40 名 SLE 儿童和 32 名健康对照者的血清样本，以测定 CXCL13 的浓度。
血清 CXCL13 浓度（pg/ml）中位数（四分位距（IQR））依次升高，包括健康对照组（71.6（66.6-81.8））、SLE 无活动疾病组（140.8（99.7-198.8），p=0.0005 与对照组相比）和活动疾病组（293.0（105.5-489.8），p=0.0001 与对照组相比）（无活动与活动；p<0.0001）。循环 CXCL13 浓度与 SLEDAI（r=0.499，p=0.029）和双链 DNA 滴度（r=0.71，p<0.001）相关。此外，有肾脏受累的患者的 CXCL13 浓度中位数高于无肾脏受累的患者（270.6（150.4-430.7）与 120.6（70.5-208.9）相比）。根据世界卫生组织狼疮肾炎病理分类，III、IV 和 V 级肾炎患儿的 CXCL13 浓度中位数高于 I 和 II 级肾炎患儿（333.9（169.4-491.5）与（180.4（107.9-209.7）相比）。
我们的数据表明，CXCL13 水平升高是 SLE 的一个特征，与疾病的活动度和严重程度相关。狼疮肾炎中 CXCL13 的表达代表了一种具有诊断和预后意义的新发病机制。CXCL13 及其受体 CXCR5 的表达的药理学调节可能是狼疮肾炎治疗的有用工具。
