a Non-Communicable Diseases Research Center , Alborz University of Medical Sciences , Karaj , Iran.
b Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital , Alborz University of Medical Sciences , Karaj , Iran.
Expert Rev Clin Immunol. 2017 Nov;13(11):1099-1106. doi: 10.1080/1744666X.2017.1384312. Epub 2017 Oct 11.
The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD).
A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications.
Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2.5%). History of autoimmunity was recorded in 125 patients during the course of the disease (26.5%). The frequency of autoimmunity in common variable immune deficiency (32.0%) was higher than other forms of PADs. The most common autoimmune manifestations were reported to be autoimmune gastrointestinal disease and autoimmune cytopenias. Among patients with autoimmunity, 87 patients (69.6%) had a history of one autoimmune disorder, while 38 patients (30.4%) had a history of multiple autoimmunities. The immune thrombocytopenic purpura and autoimmune hemolytic anemia were the most two concomitant autoimmune disorders in 16 (42.1%) of 38 patients with multiple autoimmunities. Comparing the frequency of Tregs in PAD patients with autoimmunity showed that, patients with multiple autoimmunities had lower Tregs than those with single autoimmunity (p = 0.017).
It is important that non-immunologist physicians be alert of the associated autoimmunity with PADs in order to reduce the diagnostic delay and establish timely immunoglobulin replacement therapy in these patients.
本研究旨在评估原发性抗体缺陷症（PAD）中自身免疫的频率。
本回顾性队列研究纳入了 471 名 PAD 患者。收集所有患者的人口统计学信息、临床记录和实验室数据，以调查自身免疫并发症。
11 名患者（2.5%）以免疫缺陷为首发表现出现自身免疫性疾病。在疾病过程中，125 名患者有自身免疫病史（26.5%）。普通可变免疫缺陷症（32.0%）中自身免疫的频率高于其他形式的 PAD。最常见的自身免疫表现为自身免疫性胃肠道疾病和自身免疫性血细胞减少症。在自身免疫患者中，87 名（69.6%）有 1 种自身免疫疾病史，38 名（30.4%）有多种自身免疫疾病史。在 38 名有多种自身免疫疾病史的患者中，免疫性血小板减少性紫癜和自身免疫性溶血性贫血是最常见的两种同时存在的自身免疫性疾病，分别有 16 例（42.1%）。比较自身免疫性 PAD 患者 Tregs 的频率发现，有多发性自身免疫的患者 Tregs 低于单发性自身免疫的患者（p=0.017）。
非免疫学家医生需要警惕 PAD 伴发的自身免疫，以减少诊断延误，并为这些患者及时进行免疫球蛋白替代治疗。
