Fibrillary glomerulonephritis: a rare entity with unique ultrastructural characteristics
Journal
Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia = Brazilian Journal of nephrology
Date Issued
2022-07-25
Author(s)
DOI
10.1590/2175-8239-JBN-2022-0004en
Abstract
Fibrillary glomerulonephritis (FGn) is
characterized by deposition of randomly
arranged polyclonal immune deposits
in glomerular matrix. A 56-year-old
hypertensive patient presented to our
hospital with proteinuria (3.04 g/24
hours) and an elevated serum level
of creatinine (391 μmol/L). Electron
microscopic evaluation of kidney biopsy
specimens set the diagnosis of fibrillary
glomerulonephritis (Figure 1). There was
no evidence of monoclonal components
in the blood and urine. Antinuclear and
anti-double-stranded DNA antibodies,
complement components C3 and C4, and
markers of viral hepatitis were also negative.
The benefit of immunosuppressants is
limited, and half of patients progress to
kidney failure within 2 years.
characterized by deposition of randomly
arranged polyclonal immune deposits
in glomerular matrix. A 56-year-old
hypertensive patient presented to our
hospital with proteinuria (3.04 g/24
hours) and an elevated serum level
of creatinine (391 μmol/L). Electron
microscopic evaluation of kidney biopsy
specimens set the diagnosis of fibrillary
glomerulonephritis (Figure 1). There was
no evidence of monoclonal components
in the blood and urine. Antinuclear and
anti-double-stranded DNA antibodies,
complement components C3 and C4, and
markers of viral hepatitis were also negative.
The benefit of immunosuppressants is
limited, and half of patients progress to
kidney failure within 2 years.