Anti-nephrin autoantibody positivity describes a unique subclass of podocytopathies

Autoantibodies targeting nephrin could be detected in approximately 70% of adults with untreated minimal change disease and 90% of children with idiopathic nephrotic syndrome. Anti-nephrin autoantibody presence correlated with disease activity and its presence may suggest a new subclass of podocytopathies.

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 aimed to detect anti-nephrin antibodies and included 357 adults (with diseases such as minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, IgA nephropathy, ANCA-associated 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 where this switch induced 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”, 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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Posted on 20 June 202424 June 2024