Acquired GBM Disease

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An interesting scenario of “acquired” anti-GBM disease can occur when a patient with Alport’s Syndrome gets a renal transplant.

Patients with Alport’s Syndrome–a cause of hematuria with progressive renal failure and hearing loss–have mutations in genes encoding certain subunits of the collagen IV molecule which comprises a major structural component of the glomerular basement membrane.

When they undergo a renal transplant, their immune system may well recognize the donor collagen IV as “foreign” since it has never been exposed to this antigen previously, and this can lead to the formation of circulating anti-glomerular basement membrane antibodies and subsequent allograft dysfunction.

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