Chronic renal disease in inborn errors of immunity adults patients with immunoglobulin replacement therapy

Immunoglobulin replacement therapy (IgG-RT) has changed the evolution of patients with inborn errors of immunity (IEI), significantly reducing the incidence of infections and increasing their survival to adulthood. Given the increasing survival, determining the impact of long-term IgG-RT on the development of chronic kidney disease (CKD) has become of interest.
Retrospective cohort of patients over 18 years of age in follow-up by the Immunology and Histocompatibility Unit of the Hospital C.G. Durand. We included 123 patients with requirement of IgG-RT due to IEI diagnosis.
A total of 123 patients with IEI diagnosis and IgG-RT were included. Patients were classified according to IgG-RT length: up to 10 years of treatment (YT) 17 patients (mean age 51 years), between 11 and 20 YT 24 patients (mean age 49 years), between 21 and 30 YT 16 patients (mean age 46 years) and between 31 and 40 YT 6 patients (mean age 52 years). Fifty-five percent were male, with a mean age of 48 years (27-81). Seventy-one percent were diagnosed with common variable immunodeficiency (CVID). The mean duration of IgG-RT was 17 years (2-40). Two (1.6%) cases of CKD were identified: one CVID patient with 31 years of IgG-RT and another with PI3K mutation with 8 years of IgG-RT. The first one has risk factors related to CKD. The second patient was diagnosed with lupus nephropathy. Among patients with more than 30 YT only one (16,6%) presented CKD, despite 50% also having other risk factors (dyslipidemia and arterial hypertension).
The prevalence of CKD among patients with IEI and IgG-RT is lower than in the general population but increases in the group of patients with more than 30 years of replacement therapy in coincidence with the increased frequency of risk factors.

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