Extraglomerular Vascular Involvement of Glomerulopathy with Fibronectin Deposits

Intern Med. 2021 Jul 1;60(13):2103-2107. doi: 10.2169/internalmedicine.6558-20. Epub 2021 Feb 8.

Abstract

Glomerulopathy with fibronectin deposits (GFND) is a rare hereditary kidney disease with autosomal dominant inheritance. A 21-year-old woman who had been diagnosed with GFND 10 years ago was admitted for investigation of a rapid decline in her renal function, hemolytic anemia, and cardiac dysfunction. A renal biopsy showed GFND accompanied by extraglomerular vascular lesions. Comprehensive treatments against hypertension and anemia improved the renal function. Although there have been few reports of vascular lesions in GFND, we suspect that endothelial hyperpermeability resulting from hypertension caused the fibronectin deposition and narrowing of the extraglomerular vascular lumens, thereby accelerating hypertension and inducing hemolytic anemia.

Keywords: endothelial damage; glomerulopathy with fibronectin deposits (GFND); hemolytic anemia; hypertension; vascular lesion.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Fibronectins
  • Glomerulonephritis, Membranoproliferative*
  • Humans
  • Kidney Diseases*
  • Young Adult

Substances

  • Fibronectins

Supplementary concepts

  • Glomerulopathy with fibronectin deposits