Division of Immunology, Department of Pathology, Faculty of Health Science, University of Cape Town, South Africa.
Division of Allergology and Clinical Immunology, Groote Schuur Hospital, University of Cape Town, South Africa.
Hum Immunol. 2023 May-Jul;84(5-7):337-341. doi: 10.1016/j.humimm.2023.04.004. Epub 2023 Apr 18.
Inborn errors of immunity (IEIs) or primary immunodeficiency diseases, are disorders caused by genetic defects affecting immune function. Clinically, IEI presents mainly as recurrent or severe infections, immune dysregulation (autoimmunity or autoinflammatory disorders), and lymphoproliferation with or without dysmorphic features. Humoral IEIs are the largest subgroup of IEI, with a wide spectrum of quantitative and qualitative antibody defects. These disorders are normally diagnosed based on immunological evaluation; diagnostic vaccination is part of this evaluation. This review examines the importance and relevance of diagnostic vaccination in the diagnosis of humoral IEIs and different technologies which can be utilised in diagnosis.
先天性免疫缺陷(IEI)或原发性免疫缺陷病,是由影响免疫功能的遗传缺陷引起的疾病。临床上,IEI 主要表现为反复或严重感染、免疫失调(自身免疫或自身炎症性疾病)以及伴或不伴发育异常的淋巴组织增生。体液性 IEI 是 IEI 中最大的亚组,具有广泛的定量和定性抗体缺陷。这些疾病通常根据免疫评估进行诊断;诊断性疫苗接种是该评估的一部分。本综述探讨了诊断性疫苗接种在体液性 IEI 诊断中的重要性和相关性,以及可用于诊断的不同技术。