Selective immunoglobulin A deficiency (SIgAD) is the most common primary immunodeficiency, defined as an isolated deficiency of IgA (less than 0.07 g/L). Although the majority of people born with IgA deficiency lead normal lives without significant pathology, there is nonetheless a significant association of IgA deficiency with mucosal infection, increased risks of atopic disease, and a higher prevalence of autoimmune disease. To explain these phenomena, we have performed an extensive literature review to define the geoepidemiology of IgA deficiency and particularly the relative risks for developing systemic lupus erythematosus, hyperthyroidism, hypothyroidism, type 1 diabetes mellitus, Crohn's disease, ulcerative colitis, rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, and vitiligo; these diseases have strong data to support an association. We also note weaker associations with scleroderma, celiac disease, autoimmune hepatitis, immune thrombocytopenic purpura, and autoimmune hemolytic anemia. Minimal if any associations are noted with myasthenia gravis, lichen planus, and multiple sclerosis. Finally, more recent data provide clues on the possible immunologic mechanisms that lead to the association of IgA deficiency and autoimmunity; these lessons are important for understanding the etiology of autoimmune disease.

中文翻译：

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选择性IgA缺乏症自身免疫的流行病学和临床表现。

选择性免疫球蛋白A缺乏症（SIgAD）是最常见的原发性免疫缺陷，定义为孤立的IgA缺陷（小于0.07 g / L）。尽管大多数患有IgA缺乏症的人过着正常的生活而没有明显的病理状况，但是IgA缺乏症与粘膜感染，特应性疾病的风险增加和自身免疫性疾病的患病率之间存在显着关联。为了解释这些现象，我们进行了广泛的文献综述，以明确IgA缺乏症的流行病学，特别是发展系统性红斑狼疮，甲状腺功能亢进症，甲状腺功能减退症，1型糖尿病，克罗恩病，溃疡性结肠炎，类风湿关节炎，青少年特发性疾病的相对风险关节炎，强直性脊柱炎和白癜风；这些疾病有强有力的数据来支持关联。我们还注意到与硬皮病，乳糜泻，自身免疫性肝炎，免疫性血小板减少性紫癜和自身免疫性溶血性贫血的关联较弱。与重症肌无力，扁平苔藓和多发性硬化症的关联最小，如果有关联。最后，最近的数据提供了可能导致IgA缺乏与自身免疫相关的免疫机制的线索。这些课程对于理解自身免疫病的病因很重要。最近的数据提供了可能导致IgA缺乏与自身免疫相关的免疫机制的线索；这些课程对于理解自身免疫性疾病的病因很重要。最近的数据为可能导致IgA缺乏与自身免疫相关的免疫机制提供了线索；这些课程对于理解自身免疫性疾病的病因很重要。