Primary Sjögren's syndrome (pSS) is an autoimmune disease characterised by an increased risk for non-Hodgkin lymphoma (NHL) development. Ectopic germinal centre (GC) in the salivary gland is associated with increased NHL risk in pSS, and the chemokine CXCL13 is implicated in B-cell migration and GC formation. Serum CXCL13 concentrations were quantified by ELISA in 48 healthy individuals, 273 pSS patients without NHL (pSS-nonL), and 38 pSS patients with NHL (pSS-NHL+) from the United Kingdom Primary Sjögren's Syndrome Registry cohort. PSS-nonL patients were stratified into low risk (LR), moderate risk (MR) and high risk (HR) groups according to the lymphoma risk score proposed by Fragkioudaki et al. Differences in serum CXCL13 levels among groups were analysed using the Wilcoxon method. Also, changes in serum CXCL13 over a time period of at least 1 year and a median 4 years were assessed for 200 pSS-nonL and 8 pSS-NHL+ patients. In addition, associations of serum CXCL13 with B-cell and inflammatory markers were investigated by correlation analyses and logistic regression. Serum CXCL13 levels were higher in all pSS groups compared to controls (p < 0.0001), and in pSS-NHL+ compared to pSS-nonL patients (p = 0.0204). LR patients had lower CXCL13 levels than MR patients (p < 0.0001) and pSS-NHL+ patients (p = 0.0008). CXCL13 levels remained stable over the study period for all pSS groups. CXCL13 was associated (p < 0.0005) with Immunoglobulin G (IgG), B-cell activating factor, β2 microglobulin, combined free light chains, κ and λ light chains, anti-Ro/SSA, anti-La/SSB, and erythrocyte sedimentation rate. IgG and C3 controlled for age and gender were significantly associated with NHL risk in pSS. Serum CXCL13 levels were elevated in pSS-NHL+ and MR patients compared to LR patients and remained stable over time. Further study is required to investigate the role of CXCL13 in pSS-associated NHL risk.

中文翻译：

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血清CXCL13水平与原发性干燥综合征中的淋巴瘤风险和淋巴瘤发生有关。

原发性干燥综合征（pSS）是一种自身免疫性疾病，其特征在于非霍奇金淋巴瘤（NHL）发展的风险增加。唾液腺中的异位生发中心（GC）与pSS中NHL风险增加有关，趋化因子CXCL13与B细胞迁移和GC形成有关。通过ELISA对英国原发性干燥综合征综合征登记队列中的48名健康个体，273例无NHL的pSS患者（pSS-nonL）和38例NHL的pSS患者（pSS-NHL +）进行了血清定量。根据Fragkioudaki等提出的淋巴瘤风险评分，将PSS-nonL患者分为低风险（LR），中风险（MR）和高风险（HR）组。使用Wilcoxon方法分析各组之间血清CXCL13水平的差异。也，对200名pSS-nonL和8名pSS-NHL +患者进行了至少1年和中值4年的血清CXCL13变化评估。此外，通过相关分析和逻辑回归分析了血清CXCL13与B细胞和炎症标志物的关联。与对照组相比，所有pSS组的血清CXCL13水平均较高（p <0.0001），而与pSS-nonL患者相比，pSS-NHL +组的血清CXCL13水平较高（p = 0.0204）。LR患者的CXCL13水平低于MR患者（p <0.0001）和pSS-NHL +患者（p = 0.0008）。在整个研究期间，所有pSS组的CXCL13水平均保持稳定。CXCL13与免疫球蛋白G（IgG），B细胞活化因子，β2微球蛋白，组合的自由轻链，κ和λ轻链，抗Ro / SSA，抗La / SSB和红细胞沉淀相关（p <0.0005）率。年龄和性别控制的IgG和C3与pSS中的NHL风险显着相关。与LR患者相比，pSS-NHL +和MR患者的血清CXCL13水平升高，并且随着时间的推移保持稳定。需要进一步的研究以调查CXCL13在与pSS相关的NHL风险中的作用。