Student Research Committee, Alborz University of Medical Sciences, Karaj, Iran.
Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran.
Immunol Invest. 2022 May;51(4):778-786. doi: 10.1080/08820139.2021.1872615. Epub 2021 Jan 12.
Autoimmune disorders are reported as presenting signs in patients with immunoglobulin A (IgA) deficiency. Herein, we aim to evaluate serum IgA among patients with autoimmune polyendocrinopathy.
Patients with two or more autoimmune endocrinopathies were selected and the serum IgA levels were measured. Patients with an isolated low serum IgA (<7 mg/dL) after exclusion of other causes of hypogammaglobulinemia were considered as selective IgA deficiency (SIgAD), while partial IgA deficiency (PIgAD) was defined as IgA levels below lower limits of IgA normal range for age but higher than 7 mg/dL.
Fifty-three patients (19 [35.8%] male and 34 [64.2%] female) with autoimmune polyendocrinopathy enrolled in the study. Parental consanguinity and positive family history of autoimmunity were reported in 38.0% and 52.9% of patients, respectively. Overall, IgA deficiency was observed in 5 (9.4%) patients including PIgAD in 3 (5.7%) and SIgAD in 2 (3.8%) patients. Among IgA deficient patients, the first autoimmune disorder was developed at earlier ages ( = .002), and the prevalence of infection ( = .002), lymphoproliferation ( = .021), and overlap between insulin-dependent diabetes mellitus and autoimmune thyroiditis ( = .032) were significantly higher than patients with normal IgA. Also, the number of autoimmune comorbidities was closely correlated with the occurrence of IgA deficiency ( = .008).
The prevalence of IgA deficiency in patients with autoimmune polyendocrinopathy is higher than that in the general population. In these patients, immunologic workup may lead to early diagnosis of inborn error of immunity, which can positively impact the evolution of complications and even management of the autoimmune disorders.
自身免疫性疾病被报道为免疫球蛋白 A（IgA）缺乏症患者的首发表现。在此，我们旨在评估患有自身免疫性多内分泌病的患者的血清 IgA 水平。
选择患有两种或两种以上自身免疫性内分泌病的患者，并测量其血清 IgA 水平。排除其他低丙种球蛋白血症原因后，孤立性血清 IgA 降低（<7mg/dL）的患者被认为是选择性 IgA 缺乏症（SIgAD），而部分 IgA 缺乏症（PIgAD）则定义为 IgA 水平低于年龄正常范围下限，但高于 7mg/dL。
研究共纳入 53 例（19 例男性[35.8%]和 34 例女性[64.2%]）自身免疫性多内分泌病患者。分别有 38.0%和 52.9%的患者报告存在家族遗传史和自身免疫阳性家族史。总体而言，5 例（9.4%）患者存在 IgA 缺乏症，包括 3 例（5.7%）PIgAD 和 2 例（3.8%）SIgAD。在 IgA 缺乏症患者中，首次出现自身免疫性疾病的年龄更早（ = 0.002），且感染（ = 0.002）、淋巴增生（ = 0.021）和胰岛素依赖型糖尿病与自身免疫性甲状腺炎重叠（ = 0.032）的患病率显著高于 IgA 正常的患者。此外，自身免疫性疾病合并症的数量与 IgA 缺乏症的发生密切相关（ = 0.008）。
自身免疫性多内分泌病患者的 IgA 缺乏症患病率高于普通人群。在这些患者中，免疫检查可能会导致先天性免疫缺陷的早期诊断，这可以积极影响并发症的演变，甚至自身免疫性疾病的治疗。
