Azizi Gholamreza, Ahmadi Moslem, Abolhassani Hassan, Yazdani Reza, Mohammadi Hamed, Mirshafiey Abbas, Rezaei Nima, Aghamohammadi Asghar
Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran.
Int Arch Allergy Immunol. 2016;171(3-4):180-193. doi: 10.1159/000453263. Epub 2016 Dec 24.
Primary antibody deficiencies (PADs) are the most common inherited primary immunodeficiencies in humans, characterized by hypogammaglobulinemia, an inability to produce specific antibodies, and recurrent infections mainly caused by encapsulated bacteria. However, it has been shown that inflammatory disorders, granulomatous lesions, lymphoproliferative diseases, cancer, and autoimmunity are associated with the various types of PAD. Both systemic and organ-specific autoimmune diseases could be attributed to B-cell defects in PAD patients. Immune thrombocytopenic purpura and autoimmune hemolytic anemia are the most common autoimmune disorders in this group of patients. The aim of this review is to describe the proposed mechanisms for autoimmunity and to review the literature with respect to the reported autoimmune disorders in each type of PAD.
原发性抗体缺陷（PADs）是人类最常见的遗传性原发性免疫缺陷病，其特征为低丙种球蛋白血症、无法产生特异性抗体以及主要由包膜细菌引起的反复感染。然而，已有研究表明，炎症性疾病、肉芽肿性病变、淋巴增殖性疾病、癌症和自身免疫与各种类型的PAD相关。全身性和器官特异性自身免疫性疾病都可能归因于PAD患者的B细胞缺陷。免疫性血小板减少性紫癜和自身免疫性溶血性贫血是这类患者中最常见的自身免疫性疾病。本综述的目的是描述所提出的自身免疫机制，并回顾关于每种类型PAD中报道的自身免疫性疾病的文献。
