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

Emma Materne; Baijun Zhou; Daniel DiGiacomo; Jocelyn R Farmer; Ramsay Fuleihan; Kathleen E Sullivan; Charlotte Cunningham-Rundles; Zuhair K Ballas; Daniel Suez; Sara Barmettler; USIDNET Consortium

doi:10.1016/j.jaci.2024.03.014

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

J Allergy Clin Immunol. 2024 Mar 29:S0091-6749(24)00326-9. doi: 10.1016/j.jaci.2024.03.014. Online ahead of print.

Authors

Affiliations

¹ Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, Mass; Harvard Medical School, Boston, Mass. Electronic address: ecmaterne@mgh.harvard.edu.
² Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, Mass.
³ Hackensack Meridian Health, Jersey Shore University Medical Center, Neptune, NJ.
⁴ Clinical Immunodeficiency Program, Division of Allergy and Inflammation, Beth Israel Lahey Health, Burlington, Mass.
⁵ Division of Pediatric Allergy, Immunology and Rheumatology, Columbia University, New York, NY.
⁶ Division of Allergy and Immunology, Children's Hospital of Pennsylvania, Philadelphia, Pa.
⁷ Division of Clinical Immunology, Mt. Sinai, New York, NY.
⁸ Division of Internal Medicine, Immunology, University of Iowa, Iowa City, Iowa.
⁹ DS Allergy Clinic, Irving, Tex.
¹⁰ Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, Mass; Harvard Medical School, Boston, Mass.

PMID: 38555979
DOI: 10.1016/j.jaci.2024.03.014

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.