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Fibrillary glomerulonephritis: defining the disease spectrum

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Monoclonal Gammopathies and the Kidney

Abstract

Organized fiibrillary glomerular deposits may be encountered in a variety of renal disorders, including amyloidosis, cryoglobulinemic glomerulonephritis, lupus nephritis, collagen glomerulopathies, and the entities of fiibrillary and immunotactoid glomerulonephritis. Fibrillary glomerulonephritis is a distinctive but controversial glomerulopathy fiirst reported by Rosenmann and Eliakim in 19771. This rare disorder comprises less than 1% in renal biopsy series and usually presents with renal insuffiiciency, nephrotic range proteinuria, and microhematuria2. It is characterized pathologically by the deposition in glomeruli of fiibrillary deposits that typically range from 16 to 24 nm in diameter3–6. These fiibrils usually stain for IgG and C3, with more variable and weaker positivity for other immunoglobulins2. By defiinition, the glomerular deposits are Congo-red negative, allowing their differentiation from amyloid. Hence, synonyms for this entity include ‘nonamyloidotic fiibrillary glomerulonephritis’ and ‘Congo red-negative amyloidosislike glomerulopathy’7–9.

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D’agati, V.D. et al. (2003). Fibrillary glomerulonephritis: defining the disease spectrum. In: Touchard, G., Aucouturier, P., Hermine, O., Ronco, P. (eds) Monoclonal Gammopathies and the Kidney. Springer, Dordrecht. https://doi.org/10.1007/978-94-017-0191-4_14

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  • DOI: https://doi.org/10.1007/978-94-017-0191-4_14

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-90-481-6199-7

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