Introduction: Inborn errors of immunity (IEIs) are rare inherited disorders with a broad spectrum of manifestations. Here, we aimed to delineate the atopy burden in a cohort of patients with IEIs. Methods: 313 patients with IEIs were enrolled in the study within a 9-years period, and data were collected via a questionnaire. All statistical analyses were performed using SPSS software (v. 25.0, Chicago, IL, USA). The statistical significance level was set at p #x3c; 0.05. Results: Overall, 51 out of 313 (16.3%) patients were identified to have atopic manifestations. Food allergy was detected in 34 (10.2%), atopic dermatitis in 21 (6.7%), as well as allergic asthma and allergic rhinitis each in 4 (1.3%) patients. The allergic disorders were reported as initial manifestations among 14 out of 35 (40.0%) atopic patients. Most of these 51 patients fell within the category of combined immunodeficiency (CID) (n = 38, 74.5%), followed by, severe CID (SCID) (n = 5, 9.8%), common variable immunodeficiency (n = 3, 5.9%), chronic granulomatous disease (n = 3, 5.9%), selective IgA deficiency (n = 1, 2.0%), and leukocyte adhesion defect (n = 1, 2.0%). No patient with Mendelian susceptibility to mycobacteria was found to have atopic manifestation. Atopic dermatitis (p = 0.001) and food allergy (p #x3c; 0.001) were both significantly higher in patients with CID than in other IEI groups. Among atopic patients with CID and SCID, food allergy and atopic dermatitis were the most prevalent comorbidities. Discussion/Conclusion: Atopic diseases may contribute to the clinical picture of IEIs, particularly in patients with CID. Atopy in association with other warning signs of IEIs increases the possibility of an underlying IEI.
Int Arch Allergy Immunol

中文翻译：

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313 名伊朗先天性免疫缺陷患者中特应性表现的患病率

简介：先天性免疫缺陷 (IEI) 是一种罕见的遗传性疾病，具有广泛的表现。在这里，我们旨在描述一组 IEI 患者的特应性负担。方法： 313 名 IEI 患者在 9 年内参加了这项研究，并通过问卷收集数据。所有统计分析均使用 SPSS 软件 (v. 25.0, Chicago, IL, USA) 进行。统计显着性水平设置为p #x3c；0.05。结果：总体而言，313 名患者中的 51 名 (16.3%) 被确定为具有特应性表现。食物过敏 34 例（10.2%），特应性皮炎 21 例（6.7%），过敏性哮喘和过敏性鼻炎各 4 例（1.3%）。过敏性疾病被报告为 35 名 (40.0%) 特应性患者中的 14 名的初始表现。这 51 名患者中的大多数属于联合免疫缺陷 (CID) ( n = 38, 74.5%)，其次是重度 CID (SCID) ( n = 5, 9.8%)、常见变异免疫缺陷 ( n = 3, 5.9) %)、慢性肉芽肿病 ( n = 3, 5.9%)、选择性 IgA 缺乏 ( n = 1, 2.0%) 和白细胞粘附缺陷 ( n= 1, 2.0%)。没有发现对分枝杆菌具有孟德尔易感性的患者有特应性表现。CID 患者的特应性皮炎 ( p = 0.001) 和食物过敏 ( p #x3c; 0.001) 均显着高于其他 IEI 组。在患有 CID 和 SCID 的特应性患者中，食物过敏和特应性皮炎是最常见的合并症。讨论/结论：特应性疾病可能会影响 IEI 的临床表现，尤其是在 CID 患者中。特应性与 IEI 的其他警告信号相关，增加了潜在 IEI 的可能性。
Int Arch 过敏免疫