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Anti-nephrin autoantibody positivity describes a unique subclass of podocytopathies

Presented by
Prof. Tobias B. Huber, University Medical Center Hamburg-Eppendorf, Germany
ERA 2024
Cohort study
Autoantibodies targeting nephrin could be detected in approximately 70% of adults with untreated minimal change disease and 90% of children with idiopathic nephrotic syndrome cases. Anti-nephrin autoantibody presence also correlated with disease activity.

Podocytopathies are a group of glomerular diseases in which podocyte damage leads to proteinuria. These are rare diseases with causes which are not currently fully understood [1]. The current multi-centre cohort, created to detect anti-nephrin antibodies, included 357 adults (with diseases such as minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, IgA nephropathy, ANCA glomerulonephritis and lupus nephritis), 182 children (all with idiopathic nephrotic syndrome), and 67 adults and 50 children as controls. Prof. Tobias B. Huber (University Medical Center Hamburg-Eppendorf, Germany) presented the results [2].

Anti-nephrin autoantibodies were detected in 44% of adult cases of minimal change disease, and in 9% of primary focal segmental glomerulosclerosis, but were rare in non-primary focal segmental glomerulosclerosis and membranous nephropathy, with no detections in other studied conditions or control participants. Among participants with minimal change disease, 69% of participants who were not nephrotic and did not receive immunosuppressants showed anti-nephrin autoantibody positivity. Moving to children, anti-nephrin autoantibodies were detected in 52% of cases with idiopathic nephrotic syndrome, with an increase in proportion in children who were not nephrotic and did not receive immunosuppressant therapy (90%). “Furthermore, there was a strong correlation between the detection of anti-nephrin antibodies and proteinuria, and thus disease activity”, elaborated Prof. Huber. The presenter also described several patient cases where anti-nephrin antibody detection was employed to switch immunosuppressive treatment to agents such as cyclosporin or rituximab and induce long-term remission [2].

“These results suggest a novel disease entity of anti-nephrin podocytopathy appearing in 70% of untreated minimal change disease cases and 90% of idiopathic nephrotic syndrome and anti-nephrin antibodies correlated with disease activity”, concluded Prof. Tobias B. Huber.

  1. Kopp JB, et al. Nat Rev Dis Primers. 2020;6(1):68.
  2. Huber TB, et al. Autoantibodies targeting nephrin in podocytopathies. Late Breaking Clinical Trials I, ERA 2024, 23–26 May, Stockholm, Sweden.

Medical writing support was provided by Mihai Surducan, PhD.

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