Pediatric rheumatology

Autoantibodies Targeting Nephrin in Podocytopathies

N Engl J MedPublished Multicenter cross-sectional study with experimental mouse model

Authors' conclusion

In this study, circulating antinephrin autoantibodies were common in patients with minimal change disease or idiopathic nephrotic syndrome and appeared to be markers of disease activity.

Study context

Population
Adults with glomerular diseases and children with idiopathic nephrotic syndrome, plus controls357 adults · 182 children
Participants
539 participants
Exposure
Minimal change disease vs Primary focal segmental glomerulosclerosis vs Idiopathic nephrotic syndrome vs Other conditions vs Controls
  • Minimal change diseasen = 105
  • Primary focal segmental glomerulosclerosisn = 74
  • Idiopathic nephrotic syndromen = 182
  • Controlsn = 117reference
Outcome
Prevalence of antinephrin autoantibodies
Methodological detailsmulticentre, experimental mouse model (active immunization with recombinant murine nephrin)
  • Multicentre
  • Experimental mouse model (active immunization with recombinant murine nephrin)

Main finding

As reported in the abstract

Antinephrin autoantibodies

Observational data: associations, not effects.

Further findings

Antinephrin autoantibodies in active MCD or idiopathic nephrotic syndrome without immunosuppressive treatment (subgroup)

Antinephrin autoantibody levels and disease activity · at inclusion and during follow-up

Levels correlated with disease activity

Experimental immunization in mice

Induced nephrotic syndrome, MCD-like phenotype, IgG localization to podocyte slit diaphragm, nephrin phosphorylation and severe cytoskeletal changes

Original publication

Autoantibodies Targeting Nephrin in Podocytopathies. N Engl J Med. Published 25 May 2024. doi:10.1056/nejmoa2314471

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