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选择性IgA缺乏症中特应性疾病的流行病学及临床表现

The Epidemiology and Clinical Presentations of Atopic Diseases in Selective IgA Deficiency.

作者信息

Morawska Izabela, Kurkowska Sara, Bębnowska Dominika, Hrynkiewicz Rafał, Becht Rafał, Michalski Adam, Piwowarska-Bilska Hanna, Birkenfeld Bożena, Załuska-Ogryzek Katarzyna, Grywalska Ewelina, Roliński Jacek, Niedźwiedzka-Rystwej Paulina

机构信息

Department of Clinical Immunology and Immunotherapy, Medical University of Lublin, Chodźki 4a St., 20-093 Lublin, Poland.

Department of Nuclear Medicine, Pomeranian Medical University, Unii Lubelskiej 1 St., 71-252 Szczecin, Poland.

出版信息

J Clin Med. 2021 Aug 25;10(17):3809. doi: 10.3390/jcm10173809.

Abstract

Selective IgA deficiency (sIgAD) is the most common primary immunodeficiency disease (PID), with an estimated occurrence from about 1:3000 to even 1:150, depending on population. sIgAD is diagnosed in adults and children after the 4th year of age, with immunoglobulin A level below 0.07 g/L and normal levels of IgM and IgG. Usually, the disease remains undiagnosed throughout the patient's life, due to its frequent asymptomatic course. If symptomatic, sIgAD is connected to more frequent viral and bacterial infections of upper respiratory, urinary, and gastrointestinal tracts, as well as autoimmune and allergic diseases. Interestingly, it may also be associated with other PIDs, such as IgG subclasses deficiency or specific antibodies deficiency. Rarely sIgAD can evolve to common variable immunodeficiency disease (CVID). It should also be remembered that IgA deficiency may occur in the course of other conditions or result from their treatment. It is hypothesized that allergic diseases (e.g., eczema, rhinitis, asthma) are more common in patients diagnosed with this particular PID. Selective IgA deficiency, although usually mildly symptomatic, can be difficult for clinicians. The aim of the study is to summarize the connection between selective IgA deficiency and atopic diseases.

摘要

选择性IgA缺乏症(sIgAD)是最常见的原发性免疫缺陷病(PID),根据人群不同,估计发病率约为1:3000至1:150。4岁以后的成人和儿童若免疫球蛋白A水平低于0.07g/L且IgM和IgG水平正常,则可诊断为sIgAD。通常,由于该病经常无症状,患者一生中可能都未被诊断出来。如果出现症状,sIgAD与上呼吸道、泌尿道和胃肠道更频繁的病毒和细菌感染以及自身免疫性和过敏性疾病有关。有趣的是,它还可能与其他原发性免疫缺陷病有关,如IgG亚类缺乏或特异性抗体缺乏。sIgAD很少会发展为常见变异型免疫缺陷病(CVID)。还应记住,IgA缺乏可能在其他疾病过程中出现或由其治疗引起。据推测,在诊断为此种特定原发性免疫缺陷病的患者中,过敏性疾病(如湿疹、鼻炎、哮喘)更为常见。选择性IgA缺乏症虽然通常症状较轻,但对临床医生来说可能具有挑战性。本研究的目的是总结选择性IgA缺乏症与特应性疾病之间的联系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/76eb/8432128/3e25d0daceca/jcm-10-03809-g001.jpg

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