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Chapter 7 - Glomerular Diseases with Fibrillary Deposits

from Section 2 - Glomerular Diseases

Published online by Cambridge University Press:  10 August 2023

By
Nattawat Klomjit ,
Ladan Zand  and
Mariam Priya Alexander
Edited by
Helen Liapis
Helen Liapis
Affiliation:
Ludwig Maximilian University, Nephrology Center, Munich, Adjunct Professor and Washington University St Louis, Department of Pathology and Immunology, Retired Professor
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Summary

Glomerular diseases with organized fibrillary deposits are rare entities and more so in children. They can be simply categorized as amyloid or non-amyloid diseases using the Congo red stain. All amyloidogenic proteins as a rule stain positive with Congo red. Non-amyloid diseases can be further classified into organized fibrils with immunoglobulin components (fibrillary glomerulonephritis [FGN] and immunotactoid glomerulopathy [ITG]), and those without immunoglobulin components (fibronectin glomerulopathy and collagenofibrotic glomerulopathy among others). These deposits may have overlapping features that pose a multitude of challenges in making a correct diagnosis. Routine histological features may aid in diagnosis, but electron microscopy and special stains pertaining to each disease are frequently required. Renal prognosis in these conditions remains poor and treatment options are limited.

Keywords

Fibrillary glomerulonephritisamyloidosiscollagenofibroticfibronectin glomerulopathy
Type
Chapter
Information
Pediatric Nephropathology & Childhood Kidney Tumors , pp. 159 - 170
Publisher: Cambridge University Press
Print publication year: 2023

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