The biological basis for current treatment strategies for granulomatous disease in common variable immunodeficiency.

IF 3 4区医学 Q2 ALLERGY

Current Opinion in Allergy and Clinical Immunology Pub Date : 2024-12-01 Epub Date: 2024-10-21 DOI:10.1097/ACI.0000000000001032

Astrid C van Stigt, Giulia Gualtiero, Francesco Cinetto, Virgil A S H Dalm, Hanna IJspeert, Francesco Muscianisi

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Abstract

Purpose of review: The pathogenesis of granulomatous disease in common variable immunodeficiency (CVID) is still largely unknown, which hampers effective treatment. This review describes the current knowledge on the pathogenesis of granuloma formation in CVID and the biological basis of the current treatment options.

Recent findings: Histological analysis shows that T and B cells are abundantly present in the granulomas that are less well organized and are frequently associated with lymphoid hyperplasia. Increased presence of activation markers such as soluble IL-2 receptor (sIL-2R) and IFN-ɣ, suggest increased Th1-cell activity. Moreover, B-cell abnormalities are prominent in CVID, with elevated IgM, BAFF, and CD21low B cells correlating with granulomatous disease progression. Innate immune alterations, as M2 macrophages and neutrophil dysregulation, indicate chronic inflammation. Therapeutic regimens include glucocorticoids, DMARDs, and biologicals like rituximab.

Summary: Our review links the biological context of CVID with granulomatous disease or GLILD to currently prescribed therapies and potential targeted treatments.

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常见可变免疫缺陷病肉芽肿病现行治疗策略的生物学基础。

综述的目的：常见变异性免疫缺陷病（CVID）肉芽肿病的发病机制在很大程度上仍不清楚，这妨碍了有效的治疗。本综述介绍了目前有关 CVID 肉芽肿形成发病机制的知识，以及目前治疗方案的生物学基础：组织学分析表明，T 细胞和 B 细胞大量存在于肉芽肿中，肉芽肿的组织结构较差，常伴有淋巴细胞增生。可溶性 IL-2 受体（sIL-2R）和 IFN-ɣ等活化标志物的增加表明 Th1 细胞的活性增强。此外，B 细胞异常在 CVID 中也很突出，IgM、BAFF 和 CD21 低的 B 细胞升高与肉芽肿性疾病进展相关。先天性免疫改变，如 M2 巨噬细胞和中性粒细胞失调，表明存在慢性炎症。治疗方案包括糖皮质激素、DMARDs 和生物制剂（如利妥昔单抗）。摘要：我们的综述将 CVID 伴肉芽肿病或 GLILD 的生物学背景与目前的处方疗法和潜在的靶向治疗联系起来。

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

Current Opinion in Allergy and Clinical Immunology 医学-过敏

CiteScore

5.90

自引率

3.60%

发文量

109

审稿时长

6-12 weeks

期刊介绍： This reader-friendly, bimonthly resource provides a powerful, broad-based perspective on the most important advances from throughout the world literature. Featuring renowned guest editors and focusing exclusively on one to three topics, every issue of Current Opinion in Allergy and Clinical Immunology delivers unvarnished, expert assessments of developments from the previous year. Insightful editorials and on-the-mark invited reviews cover key subjects such as upper airway disease; mechanisms of allergy and adult asthma; paediatric asthma and development of atopy; food and drug allergies; and immunotherapy.