Clinical Immunology, Reference Center for Rare Diseases, Faculdade de Medicina, Centro Universitario Saude ABC, Avenida Lauro Gomes, 2000, Santo Andre, SP, 09060-870, Brazil.
Curr Allergy Asthma Rep. 2020 Jul 11;20(10):59. doi: 10.1007/s11882-020-00957-x.
The aim of this review is as follows: (1) to present the role of otitis as a warning sign for inborn errors of immunity (IEI), (2) to establish which patients presenting otitis should be investigated for IEI, (3) to review data about main IEI associated with otitis-prone patients.
Otitis media is a very common infection in general population. The concept of otitis-prone children established a certain frequency of the infections in order to look for conditions leading to them. The confirmation of middle ear impairment by specialists has demonstrated better confiability. The hallmarks for immunologic evaluation are the presence of complications as mastoiditis and membrane perforation, failure to thrive, and additional respiratory symptoms. Humoral immunodeficiencies have been more frequently described in association with otitis-prone patients, for example, hypogammaglobulinemia, MBL deficiency, and IEI associated with major syndromes. Most of the patients with confirmed IEI present otitis as one of the recurrent infections. It is suggested the investigation of immune defects in patients with otitis, and the following warning signs are suggested: Otitis evolving with mastoiditis, abscesses, or systemic infections; no response to appropriate antibiotic therapy; otitis media associated with other infections; recurrent otitis leading to failure to thrive and general developmental delay; and family history of primary immunodeficiency and/or consanguinity.
本文旨在：(1)阐述中耳炎作为固有免疫缺陷（IEI）的警示信号的作用；(2)确定哪些出现中耳炎的患者应进行 IEI 检查；(3)回顾与易患中耳炎患者相关的主要 IEI 的相关数据。
中耳炎在普通人群中是一种非常常见的感染。易患中耳炎儿童的概念确立了一定的感染频率，以便寻找导致感染的情况。专家对中耳损伤的确认显示出更好的可靠性。免疫评估的特征是存在并发症，如乳突炎和鼓膜穿孔、生长不良和额外的呼吸道症状。体液免疫缺陷与易患中耳炎的患者更频繁地相关联，例如低丙种球蛋白血症、MBL 缺乏和与主要综合征相关的 IEI。大多数确诊为 IEI 的患者都将中耳炎作为反复感染之一。建议对中耳炎患者进行免疫缺陷检查，并提出以下警示信号：伴有乳突炎、脓肿或全身感染的中耳炎进展；对适当的抗生素治疗无反应；与其他感染相关的中耳炎；导致生长不良和整体发育迟缓的复发性中耳炎；以及原发性免疫缺陷和/或近亲结婚的家族史。
