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. 2024 Jul;154(1):237-242.e1.
doi: 10.1016/j.jaci.2024.03.014. Epub 2024 Mar 29.

Renal complications in patients with predominantly antibody deficiency in the United States Immune Deficiency Network (USIDNET)

Emma Materne  1 , Baijun Zhou  2 , Daniel DiGiacomo  3 , Jocelyn R Farmer  4 , Ramsay Fuleihan  5 , Kathleen E Sullivan  6 , Charlotte Cunningham-Rundles  7 , Zuhair K Ballas  8 , Daniel Suez  9 , Sara Barmettler  10 ; USIDNET Consortium
Affiliations

Renal complications in patients with predominantly antibody deficiency in the United States Immune Deficiency Network (USIDNET)

Emma Materne et al. J Allergy Clin Immunol. 2024 Jul.

Abstract

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.

Keywords: Primary immunodeficiency; chronic kidney disease; common variable immunodeficiency; immunophenotype; inborn errors of immunity; nephritis; predominantly antibody deficiency; renal disease.

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Conflict of interest statement

Disclosure of potential conflict of interest: J. R. Farmer is an ongoing consultant for Pharming and has received investigator-initiated research grants from Pfizer, Bristol-Myers Squibb, and Pharming with no direct relation to the work presented. The rest of the authors declare that they have no relevant conflicts of interest.

Figures

FIG 1.
FIG 1.
Prevalence of renal complications in patients with PAD. For each renal condition, prevalence is reported as number and percentage above the bar on the graph.
FIG 2.
FIG 2.
Immunophenotype in the overall PAD cohort versus in patients with any renal complication (A), nephritis (B), and CKD (C). B-cell subsets in the cohort of those with PAD versus in patients with any renal complication (D) and CKD (B). Immunoglobulin (IgG, IgA, IgM, and IgE) levels in the cohort of those with PAD versus in patients with any renal complication (F), nephritis (G), and CKD (H). *P < .05.
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