ArticleKidney medicine2026
Fibrillary Glomerulonephritis and Apolipoprotein A-IV Amyloidosis: A Rare Dual Pathology Diagnosed by Mass Spectrometry and DNAJB9 Immunohistochemistry.
Article in Kidney medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Fibrillary glomerulonephritis is a rare cause of proteinuric kidney disease characterized by Congo red-negative fibrillary deposits and typically shows DNAJB9 positivity on immunohistochemistry. Amyloidosis is defined by Congo red positivity and can be typed by laser microdissection-tandem mass spectrometry when routine studies are inconclusive. We report the case of a 64-year-old man with proteinuria and declining kidney function whose kidney biopsy showed DNAJB9-positive fibrillary glomerulonephritis in glomeruli, but Congo red-positive deposits confined to the medulla were DNAJB9 negative. Laser microdissection-tandem mass spectrometry of the medullary deposits identified apolipoprotein A-IV amyloidosis, establishing concurrent fibrillary glomerulonephritis and apolipoprotein A-IV amyloidosis in the same biopsy. Apolipoprotein A-IV amyloidosis is often medullary predominant and, in rare hereditary forms related to autosomal dominant
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