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1 results

  • Chapter 7 - Glomerular Diseases with Fibrillary Deposits

  • from Section 2 - Glomerular Diseases
  • Edited by Helen Liapis, Ludwig Maximilian University, Nephrology Center, Munich, Adjunct Professor and Washington University St Louis, Department of Pathology and Immunology, Retired Professor
  • Book:
    Pediatric Nephropathology & Childhood Kidney Tumors
    Published online:
    10 August 2023
    Print publication:
    09 March 2023, pp 159-170

  • 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.