背景:先前的研究报道,肾功能不全发生在一小部分主要为抗体缺乏症(PAD)的患者和约2%的常见可变免疫缺陷症(CVID)的患者中。
目的:我们的研究目的是了解和评估美国免疫缺陷网络(USIDNET)队列中PAD患者肾脏并发症的患病率和类型。我们假设某些肾脏并发症与PAD患者免疫表型的严重程度之间存在关联。
方法:我们对患有肾脏并发症的USIDNET队列中的PAD患者进行了查询。有肾脏疾病的患者,如慢性肾脏疾病(CKD),肾结石,肾炎,和肾功能衰竭综合征被包括在内。我们比较了免疫表型,流式细胞术和免疫球蛋白(Ig)水平的PAD患者肾脏并发症的总PADUSIDNET队列。
结果:我们发现140/2071(6.8%)的PAD患者有肾脏并发症。这50人(35.7%)患有CKD,46人(32.9%)患有肾结石,18人(12.9%)有肾炎,50例(35.7%)有其他肾脏并发症。与PAD患者的总USIDNET队列相比,CKD患者的ALCs较低,CD3+T细胞,CD4+T细胞,CD19+B细胞,CD20+B-细胞和CD27+IgA-B-细胞(全部p<0.05)。肾炎患者的ALC较低,CD19+B细胞,与没有肾病的PAD患者相比,CD27+B细胞和IgE水平(全部p<0.05)。
结论:我们发现USIDNET队列中有6.8%的PAD患者有肾脏并发症。与整个PAD队列相比,患有肾炎和CKD的患者具有更严重的免疫表型。
BACKGROUND: Prior studies have reported that renal insufficiency occurs in a small percentage of patients with predominantly antibody deficiency (PAD) and in about 2% of patients with common variable immunodeficiency.
OBJECTIVE: The goal of our study was to understand and evaluate the prevalence and type of renal complications in patients with PAD in the United States Immunodeficiency Network (USIDNET) cohort. We hypothesized that there is an association between certain renal complications and severity of immunophenotype in patients with PAD.
METHODS: We performed a query of patients with PAD from the USIDNET cohort with renal complications. Patients with documented renal disease such as chronic kidney disease (CKD), nephrolithiasis, nephritis, and renal failure syndrome were included. We compared immunophenotype, flow cytometry findings, and immunoglobulin levels of patients with PAD accompanied by renal complications with those of the total USIDNET cohort of patients with PAD.
RESULTS: We determined that 140 of 2071 patients with PAD (6.8%) had renal complications. Of these 140 patients, 50 (35.7%) had CKD, 46 (32.9%) had nephrolithiasis, 18 (12.9 %) had nephritis, and 50 (35.7%) had other renal complications. Compared with the total USIDNET cohort of patients with PAD, patients with CKD had lower absolute lymphocyte counts, CD3+ T-cell counts, CD4+ T-cell counts, CD19+ B-cell counts, CD20+ B-cell counts, and CD27+IgD- B-cell counts (P < .05 for all). Patients with nephritis had lower absolute lymphocyte counts, CD19+ B-cell counts, CD27+ B-cell counts, and IgE levels (P < .05 for all) than patients with PAD without renal disease.
CONCLUSIONS: We determined that 6.8% of the USIDNET cohort of patients with PAD had a documented renal complication. Compared with the overall cohort of patients with PAD, those patients with nephritis and CKD had a more severe immunophenotype.