Fibrillary Glomerulonephritis with Splenic Involvement: A Detailed Autopsy Study

Fibrillary glomerulonephritis (FGN) is characterized by the deposition of IgG-positive, randomly arranged, nonbranching, non-Congophilic fibrils in the glomeruli. The possibility of multiorgan involvement, as in amyloidosis, has been raised. The authors report the first detailed autopsy study on a p...

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Bibliographic Details
Published in:Ultrastructural pathology 2008-01, Vol.32 (3), p.113-121
Main Authors: Satoskar, Anjali A., Calomeni, Edward, Nadasdy, Gyongyi, Tozbikan, Gary, Hitchcock, Charles, Hebert, Lee, Nadasdy, Tibor
Format: Article
Language:English
Subjects:
Autopsy
autopsy, colloidal gold immunoelectron microscopy, fibrillary glomerulonephritis, immunofluorescence microscopy, non-Congophilic fibrils
Female
Fluorescent Antibody Technique
Glomerulonephritis - complications
Glomerulonephritis - pathology
Glomerulonephritis - physiopathology
Gold Colloid
Humans
Immunoglobulin kappa-Chains - metabolism
Kidney Glomerulus - ultrastructure
Microscopy, Immunoelectron
Middle Aged
Splenic Diseases - complications
Splenic Diseases - pathology
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Summary:Fibrillary glomerulonephritis (FGN) is characterized by the deposition of IgG-positive, randomly arranged, nonbranching, non-Congophilic fibrils in the glomeruli. The possibility of multiorgan involvement, as in amyloidosis, has been raised. The authors report the first detailed autopsy study on a patient with FGN, with thorough examination of the organs by electron microscopy, colloidal gold immunoelectron microscopy, and immunofluorescence staining. Thin, wavy fibrils (extracellular matrix filaments) ranging from 6 to 17 nm were seen in all other organs, but only kidney and spleen showed the typical rigid, nonbranching fibrils of FGN with specific gold label. This study suggests that FGN is mainly a renal-limited disease with possible involvement of the spleen.
ISSN:0191-3123
1521-0758
DOI:10.1080/01913120801937723