Recurrent C3 Glomerulonephritis with an ADAMTS 13 Gene Variant: A Case Report and Literature Review.

PMID 35946293 | DOI 10.4103/1319-2442.352441 · Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia · 2021 · View on PubMed ↗

C3 glomerulonephritis (C3GN) is a recently described form of GN that mainly occurs in children and young adults. It results from dysregulation of the alternative complement pathway. Studies have shown that dense deposit disease has a high recurrence rate; however, since C3GN is a recently described disorder, its recurrence rate is still variable. A 28-year-old male with end-stage renal disease caused by C3GN underwent renal transplantation. After 19 months, the patient experienced recurrent C3GN (rC3GN) that involved a variant of unknown significance in the ADAMTS13 gene. Over a short span of time, the patient suffered from rapid deterioration of the graft function that required renal replacement therapy. This is the first case of rC3GN that possibly involved genetic alteration, a variant within the ADAMTS 13 gene.