CXCL13 as a Biomarker of Complex Common Variable Immunodeficiency.

IF 4.1 2区医学 Q1 IMMUNOLOGY

Journal of Clinical Immunology Pub Date : 2025-11-25 DOI:10.1007/s10875-025-01963-2

Ioasaf Karafotias, Helene Martini, Charlotte V Lee, Terrence T J Hunter, Padmalal Gurugama, Mary Guckian, Rachael Steven, Stephen Jolles, Mark Peakman, David Fear, Mohammad A A Ibrahim

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引用次数: 0

Abstract

Background: Common Variable Immunodeficiency (CVID) is a group of heterogeneous disorders with common denominators of impaired antibody production and function, and recurrent infections. Currently, prognostic biomarkers for CVID are limited. CXCL13 is a critical regulator of germinal centre responses and antibody production, with T follicular helper (Tfh) cells as a major source, and acts as a potent B cell chemoattractant. Serum levels of CXCL13 are increased in chronic inflammatory conditions and malignancy.

Objectives: We aimed to explore whether serum CXCL13 levels are altered in CVID and whether they can categorise the patients based on their clinical and immune phenotype.

Methods: We compared the serum levels of CXCL13 between CVID and healthy donors (HD) and associated them with the clinical and immune phenotype of the patients.

Results: The serum levels of CXCL13 were higher in CVID, especially in female patients, as compared to HD, and were positively correlated with the number of clinical complications in CVID and the total peripheral circulating Tfh cells (cTfh). CVID patients with higher levels of CXCL13 were more likely to have clinical complications and/or high frequency of CD21^low B cells or low frequency of switched memory B cells.

Conclusions: CXCL13 can categorise heterogeneous patients with CVID and be used as a biomarker of complex disease.

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CXCL13作为复杂共同变量免疫缺陷的生物标志物。

背景：共同可变免疫缺陷（CVID）是一组异质性疾病，具有抗体产生和功能受损以及复发性感染的共同特征。目前，CVID的预后生物标志物有限。CXCL13是生发中心反应和抗体产生的关键调节因子，以T滤泡辅助细胞（Tfh）为主要来源，并作为一种有效的B细胞化学引诱剂。血清CXCL13水平在慢性炎症和恶性肿瘤中升高。目的：我们旨在探讨血清CXCL13水平是否在CVID中发生改变，以及它们是否可以根据患者的临床和免疫表型对患者进行分类。方法：比较CVID和健康供者（HD）的血清CXCL13水平，并将其与患者的临床和免疫表型联系起来。结果：CVID患者血清CXCL13水平高于HD患者，尤其是女性患者，且与CVID临床并发症数及外周血Tfh细胞总量（cTfh）呈正相关。CXCL13水平较高的CVID患者更容易出现临床并发症和/或CD21low B细胞频率高或开关记忆B细胞频率低。结论：CXCL13可以对异质性CVID患者进行分类，并可作为复杂疾病的生物标志物。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Clinical Immunology 医学-免疫学

CiteScore

12.20

自引率

9.90%

发文量

218

审稿时长

2 months

期刊介绍： The Journal of Clinical Immunology publishes impactful papers in the realm of human immunology, delving into the diagnosis, pathogenesis, prognosis, or treatment of human diseases. The journal places particular emphasis on primary immunodeficiencies and related diseases, encompassing inborn errors of immunity in a broad sense, their underlying genotypes, and diverse phenotypes. These phenotypes include infection, malignancy, allergy, auto-inflammation, and autoimmunity. We welcome a broad spectrum of studies in this domain, spanning genetic discovery, clinical description, immunologic assessment, diagnostic approaches, prognosis evaluation, and treatment interventions. Case reports are considered if they are genuinely original and accompanied by a concise review of the relevant medical literature, illustrating how the novel case study advances the field. The instructions to authors provide detailed guidance on the four categories of papers accepted by the journal.