Freely Filtered 042: Nephrin Antibodies Cause Minimal Change Disease. Wait...What?

Minimal change disease has classically been described as having negative immunofluorescence and to not be antibody mediated. But here comes Dr Weins with a fresh study smashing this orthodoxy. She proposes anti-nephrin antibodies as the etiologic agent driving minimal change disease.

The Filtrate:

Joel Topf

Jennie Lin

Joshua Waitzman

Nayan Arora

Sophia Ambruso

Special Guest:

Astrid Weins (@AstridWeins) renal pathologist at the Brigham and Woman’s Hospital and Harvard Medical School

Editor:

Nayan Aurora

Show Notes:

Summary of minimal change disease according to the orthodoxy: Light microscopy of renal biopsy specimens from patients with MCD shows minimal to no change; however, electron microscopy reveals the effacement of podocyte foot processes. To add to the conundrum, immunofluorescent staining of biopsy specimens is negative, and no immune complexes are evident.

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Most of these patients have a homozygous truncating mutation (Fin-major mutation) in the nephrin gene (NPHS1), leading to total absence of the major podocyte protein, nephrin. After RTx, these patients develop anti-nephrin antibodies resulting in nephrotic range proteinuria.

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