Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Medical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
Trends Immunol. 2017 Mar;38(3):206-216. doi: 10.1016/j.it.2016.11.008. Epub 2016 Dec 22.
Common variable immunodeficiency (CVID) is an immune disorder that not only causes increased susceptibility to infection, but also to inflammatory complications such as autoimmunity, lymphoid proliferation, malignancy, and granulomatous disease. Recent findings implicate the microbiome as a driver of this systemic immune dysregulation. Here, we critically review the current evidence for a role of the microbiome in the pathogenesis of CVID immune dysregulation, and describe the possible immunologic mechanisms behind causes and consequences of microbial dysbiosis in CVID. We integrate this evidence into a model describing a role for the gut microbiota in the maintenance of inflammation and immune dysregulation in CVID, and suggest research strategies to contribute to the development of new diagnostic tools and therapeutic targets.
普通变异性免疫缺陷（CVID）是一种免疫紊乱疾病，不仅会导致感染易感性增加，还会导致炎症并发症，如自身免疫、淋巴增生、恶性肿瘤和肉芽肿病。最近的研究结果表明，微生物组是导致这种全身免疫失调的驱动因素。在这里，我们批判性地回顾了微生物组在 CVID 免疫失调发病机制中的作用的现有证据，并描述了 CVID 中微生物失调的原因和后果背后可能的免疫机制。我们将这些证据整合到一个模型中，描述了肠道微生物群在维持 CVID 炎症和免疫失调中的作用，并提出了研究策略，以促进新的诊断工具和治疗靶点的发展。
