Fibronectin glomerulopathy in a kidney allograft biopsy

Nathaniel T Klair, Salman B. Mahmood, Rasha El-Rifai, Cynthia C. Nast, Lihong Bu, Adam Bregman

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Fibronectin glomerulopathy is a rare genetic nephropathy with only a few cases of post-transplant recurrence being reported previously. We highlight a case that was initially misdiagnosed and emphasize the importance of full immunofluorescence and electron microscopy evaluation in allograft biopsies. Case presentation: A 36-year-old male with a history of end-stage kidney disease secondary to biopsy-proven type 1 membranoproliferative glomerulonephritis (MPGN) status-post living unrelated donor kidney transplant 12 years prior, presented with increasing creatinine and proteinuria. Biopsy was performed and was consistent with fibronectin glomerulopathy. Subsequent genetic testing revealed an FN1 mutation, the primary gene associated with this condition. Conclusions: Full histologic evaluation of the allograft biopsy corrected the diagnosis and additionally suggested that the patient's mother, who had expired in her 30s and had received a diagnosis of type 1 MPGN on autopsy, likely also had fibronectin glomerulopathy, enabling appropriate genetic counseling for the family.

Original languageEnglish (US)
Article number359
JournalBMC Nephrology
Volume24
Issue number1
DOIs
StatePublished - Dec 2023

Bibliographical note

Publisher Copyright:
© 2023, The Author(s).

Keywords

  • Fibronectin glomerulopathy
  • FN1 mutation
  • Kidney biopsy

PubMed: MeSH publication types

  • Case Reports
  • Journal Article

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