The purpose of this study is to describe both clinical and immunological features in large cohort of adult patients with IgG subclass deficiency, and response to immunoglobulin therapy. This is a retrospective study of data obtained from electronic medical records and paper charts of 78 patients with IgG subclass deficiency seen and followed at our immunology clinics from 2010 to 2016. Both isolated selective IgG subclass deficiency as well as combined (two) subclass deficiencies were observed. IgG3 subclass deficiency, isolated and in combination with other IgG subclass deficiency, is the most frequent of IgG subclass deficiency. A majority of patients presented with upper and lower respiratory tract infections, especially chronic sinusitis. Both allergic and autoimmune manifestations are common; however, there is no subclass preference. The proportions and absolute numbers of CD3+ T cells, CD4+ T and CD8+ T cells, CD19+ B cells, and CD3-CD16+CD56+ NK cells were normal in the majority of patients in all IgG subclass deficiencies. Total serum IgG levels did not correlate with IgG subclass levels across all IgG subclass deficiencies. Anti-pneumococcal polysaccharide antibody responses were impaired in 56% of patients. IgG3 subclass deficiency is the most common IgG subclass deficiency, and anti-polysaccharide antibody responses are distributed among IgG subclasses with modest preference in IgG2 subclass. The majority of patients treated with immunoglobulin responded by reduction in frequency of infections and requirement of antibiotics.

中文翻译：

---

78例原发性选择性IgG亚类缺陷成人患者的临床和免疫学特征。

这项研究的目的是描述大群患有IgG亚类缺陷的成年患者的临床和免疫学特征，以及对免疫球蛋白治疗的反应。这是一项回顾性研究，从电子病历和纸质图表获得的数据，这些数据从2010年至2016年在我们的免疫学诊所就诊并随访了78例IgG亚类缺陷患者。观测到的。IgG3亚类缺乏症与其他IgG亚类缺乏症相分离并结合在一起，是IgG亚类缺乏症最常见的情况。大多数患者出现上呼吸道和下呼吸道感染，尤其是慢性鼻窦炎。变态反应和自身免疫表现都很常见。但是，没有子类首选项。在所有IgG亚型缺陷的大多数患者中，CD3 + T细胞，CD4 + T和CD8 + T细胞，CD19 + B细胞和CD3-CD16 + CD56 + NK细胞的比例和绝对数量均正常。在所有IgG亚类缺陷中，血清总IgG水平与IgG亚类水平不相关。56％的患者抗肺炎球菌多糖抗体反应受损。IgG3亚类缺陷是最常见的IgG亚类缺陷，并且抗多糖抗体反应分布在IgG2亚类中，在IgG2亚类中有中等偏爱。接受免疫球蛋白治疗的大多数患者通过降低感染频率和抗生素需求来应对。在所有IgG亚类缺陷的大多数患者中，CD3 + CD16 + CD56 + CD56 + NK细胞均正常。在所有IgG亚类缺陷中，血清总IgG水平与IgG亚类水平不相关。56％的患者抗肺炎球菌多糖抗体反应受损。IgG3亚类缺陷是最常见的IgG亚类缺陷，并且抗多糖抗体反应分布在IgG2亚类中，在IgG2亚类中有中等偏爱。接受免疫球蛋白治疗的大多数患者通过降低感染频率和抗生素需求来应对。在所有IgG亚类缺陷的大多数患者中，CD3 + CD16 + CD56 + CD56 + NK细胞均正常。在所有IgG亚类缺陷中，血清总IgG水平与IgG亚类水平不相关。56％的患者抗肺炎球菌多糖抗体反应受损。IgG3亚类缺陷是最常见的IgG亚类缺陷，并且抗多糖抗体反应分布在IgG2亚类中，在IgG2亚类中有中等偏爱。接受免疫球蛋白治疗的大多数患者通过降低感染频率和抗生素需求来应对。IgG3亚类缺陷是最常见的IgG亚类缺陷，并且抗多糖抗体反应分布在IgG2亚类中，在IgG2亚类中有中等偏爱。接受免疫球蛋白治疗的大多数患者通过降低感染频率和抗生素需求来应对。IgG3亚类缺陷是最常见的IgG亚类缺陷，并且抗多糖抗体反应分布在IgG2亚类中，在IgG2亚类中有中等偏爱。接受免疫球蛋白治疗的大多数患者通过降低感染频率和抗生素需求来应对。