Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, Mass; Harvard Medical School, Boston, Mass.
Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, Mass.
J Allergy Clin Immunol. 2024 Jul;154(1):237-242.e1. doi: 10.1016/j.jaci.2024.03.014. Epub 2024 Mar 29.
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.
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.
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.
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 CD27IgD 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.
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.
先前的研究报告表明,主要抗体缺陷(PAD)患者中仅有一小部分会出现肾功能不全,而普通可变免疫缺陷患者中约有 2%会出现肾功能不全。
我们研究的目的是了解和评估美国免疫缺陷网络(USIDNET)队列中 PAD 患者的肾脏并发症的患病率和类型。我们假设 PAD 患者的某些肾脏并发症与免疫表型严重程度之间存在关联。
我们对 USIDNET 队列中伴有肾脏并发症的 PAD 患者进行了查询。纳入有肾脏疾病病史的患者,如慢性肾脏病(CKD)、肾结石、肾炎和肾衰竭综合征。我们比较了伴有肾脏并发症的 PAD 患者的免疫表型、流式细胞术结果和免疫球蛋白水平与 PAD 总 USIDNET 队列患者的差异。
我们确定在 2071 例 PAD 患者中,有 140 例(6.8%)发生了肾脏并发症。在这 140 例患者中,50 例(35.7%)患有 CKD,46 例(32.9%)患有肾结石,18 例(12.9%)患有肾炎,50 例(35.7%)患有其他肾脏并发症。与 PAD 总 USIDNET 队列患者相比,CKD 患者的绝对淋巴细胞计数、CD3 T 细胞计数、CD4 T 细胞计数、CD19 B 细胞计数、CD20 B 细胞计数和 CD27IgD B 细胞计数均较低(所有 P 值均<.05)。与无肾脏疾病的 PAD 患者相比,肾炎患者的绝对淋巴细胞计数、CD19 B 细胞计数、CD27 B 细胞计数和 IgE 水平均较低(所有 P 值均<.05)。
我们确定 USIDNET 队列中 6.8%的 PAD 患者有明确的肾脏并发症。与 PAD 总体队列患者相比,伴有肾炎和 CKD 的患者免疫表型更为严重。