ANTI-NEPHRIN ANTIBODIES IN A LARGE COHORT OF PATIENTS WITH IDIOPATHIC NEPHROTIC SYNDROME

Introduction:

Circulating anti-NEPHRIN antibodies have been proposed as responsible for the disarrangement of the slit diaphragm architecture in patients with idiopathic nephrotic syndrome (INS). However, available publications included limited number of cases and controls, focused on adults, and used multiple assays for antibody detection, preventing comparisons across studies.

Methods:

We measured circulating anti-NEPHRIN antibodies in 302 subjects, including 203 INS patients, 32 kidney transplant recipients with or without FSGS recurrence and 63 controls. For a subset of 185 patients, we also had serial samples (total serum samples: 425). We compared the performance of three ELISA assays to quantify anti-NEPHRIN IgG, targeting different extracellular domains.

Results:

Overall, serum levels of anti-NEPHRIN antibodies were significantly higher in INS patients compared to controls. However, circulating anti-NEPHRIN antibodies showed no correlation with levels of proteinuria, histological classification, or FSGS recurrence after kidney transplant. Circulating anti-NEPHRIN antibodies declined following anti-CD20 infusion, but the serum levels did not correlate with INS relapses.

Conclusions:

Patients with INS have high levels of circulating anti-NEPHRIN antibodies, but we found no association between anti-NEPHRIN antibodies and disease activity. While our data are in line with the potential pathogenic role of these antibodies, they do not support their widespread use for monitoring disease activity.

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.