普通变异性免疫缺陷与 IgG 缺乏症的鉴别。
Differentiation of Common Variable Immunodeficiency From IgG Deficiency.
机构信息
The Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY; The Division of Clinical Immunology and Allergy, Department of Medicine, Maisonneuve-Rosemont Hospital, University of Montreal, Montreal, Quebec, Canada.
The Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY; The Division of Allergy and Clinical Immunology, Department of Medicine, Darmouth-Hitchcock Concord Medical Center, Concord, NH.
出版信息
J Allergy Clin Immunol Pract. 2019 Apr;7(4):1277-1284. doi: 10.1016/j.jaip.2018.12.004. Epub 2018 Dec 14.
BACKGROUND
Common variable immunodeficiency (CVID) and IgG deficiency are 2 of the more prevalent primary humoral immune defects. The former is defined by consensus with criteria for quantitative and qualitative antibody defects, whereas the latter is used to describe patients with reduced IgG, who commonly have recurrent sinopulmonary infections but do not fulfill CVID criteria. However, these patients are often given this diagnosis.
OBJECTIVE
To compare immunologic findings and clinical manifestations of 2 large cohorts of patients with CVID or IgG deficiency to better delineate differences between these syndromes.
METHODS
We extracted clinical and laboratory data from electronic medical records of patients at our institution who had received International Classification of Disease codes for either CVID, or IgG deficiency. We gathered immunoglobulin levels, lymphocyte subpopulation counts, and serological vaccine responses. In some patients, we performed flow cytometry to determine percentages of memory and switched-memory B cells. We compiled and statistically compared clinical data related to infectious manifestations, bronchiectasis, autoimmune diseases, infiltrative inflammatory processes, and lymphoid malignancies.
RESULTS
In contrast to IgG-deficient patients, we found that patients with CVID had lower IgG levels, greater unresponsiveness to most vaccines, lower percentages of memory and isotype switched-memory B cells, and lower CD4 T-cell counts. Clinically, patients with CVID presented similar rates of sinusitis and pneumonias, but a significantly higher prevalence of bronchiectasis and especially noninfectious complications.
CONCLUSIONS
CVID and IgG deficiency do not share the same disease spectrum, the former being associated with immunodysregulative manifestations and markers of a more severe immune defect. These data may allow clinicians to distinguish these conditions and the management differences that these patients pose.
背景
普通变异型免疫缺陷（CVID）和 IgG 缺乏症是两种更为常见的原发性体液免疫缺陷。前者通过共识和定量及定性抗体缺陷的标准定义，而后者用于描述 IgG 减少的患者，这些患者常患有复发性鼻窦和肺部感染，但不符合 CVID 标准。然而，这些患者通常被给予该诊断。
目的
比较 CVID 或 IgG 缺乏症的 2 大患者队列的免疫学发现和临床表现，以更好地区分这些综合征之间的差异。
方法
我们从我们机构的患者电子病历中提取了患有 CVID 或 IgG 缺乏症的患者的临床和实验室数据。我们收集了免疫球蛋白水平、淋巴细胞亚群计数和血清学疫苗反应。在一些患者中，我们进行了流式细胞术以确定记忆和转换记忆 B 细胞的百分比。我们整理并统计比较了与感染表现、支气管扩张、自身免疫性疾病、浸润性炎症过程和淋巴样恶性肿瘤相关的临床数据。
结果
与 IgG 缺乏症患者相比，我们发现 CVID 患者的 IgG 水平更低，对大多数疫苗的反应性更差，记忆和同种型转换记忆 B 细胞的百分比更低，CD4 T 细胞计数更低。临床上，CVID 患者的鼻窦炎和肺炎发生率相似，但支气管扩张的发生率明显更高，尤其是非传染性并发症。
结论
CVID 和 IgG 缺乏症并不具有相同的疾病谱，前者与免疫失调表现和更严重的免疫缺陷标志物相关。这些数据可能使临床医生能够区分这些疾病，并了解这些患者所带来的管理差异。
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