We describe the case of a 67 year-old female who presented weakness and fatigue. Laboratory data showed nephrotic level of proteinuria and dyslipidemia. A renal biopsy was performed, and studied by light microscopy, immuno-fluorescence and electron microscopy. Ultra-structural analysis revealed the existence of organized fibrillary deposits, straight and without ramifications, the thickness of which ranged from 15 to 20 nm. These fibres were identified, by light microscopy, as slightly nodular mesangial expansions PAS positive, Congo red negative and weakly positive for IgG. Given the above findings, the diagnosis was fibrillary glomerulonephritis. Glomerular lesions with organized deposits may exhibit syndromic and pathological overlap. For this reason it is important to initially discriminate between positive and negative Congo red deposits, using, in the latter case, transmission electron microscopy to distinguish between immuno-tactoid and fibrillary glomerulonephritis. This differentiation relies not only on ultrastructural features, but on different clinical characteristics. Unlike what happens with fibrillary glomerulonephritis, the immuno-tactoid shows a strong association with lymphoproliferative processes.
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Cureus
December 2024
Nephrology Department, Unidade Local de Saúde (ULS) Algarve - Hospital de Faro, Faro, PRT.
Fibrillary glomerulonephritis (FGN) is a rare glomerular disease characterized by the deposition of fibrils within the mesangium and glomerular basement membrane. Most cases are idiopathic, but it can be linked to autoimmune diseases, neoplasms, and infections. There is limited evidence on the best treatment approach, and many patients progress to end-stage kidney disease.
View Article and Find Full Text PDFBr J Haematol
January 2025
Department of Renal Medicine, University Hospital Birmingham NHS Trust, Birmingham, UK.
This guideline provides consensus opinion on the investigations required for people presenting with suspected monoclonal gammopathy of renal significance to both nephrology and haematology physicians. The guideline discusses the principles of treating a patient with MGRS and provides recommendations for both supportive management and haematological therapy. It details the recommended on-going monitoring required for both specialty areas.
View Article and Find Full Text PDFCureus
October 2024
Nephrology, University of California, Los Angeles (UCLA) Kern Medical, Bakersfield, USA.
Fibrillary glomerulonephritis is a rare glomerular disease with some correlations between this condition alongside viral infections, malignancy, and autoimmune pathologies. However, the question regarding the pathogenesis is whether patients who develop fibrillary glomerulonephritis do so irrespective of these other pathologies or whether such pathologies induce fibrillary glomerulonephritis through an unknown mechanism. As such, attempts have been made to create associations with this disease to demonstrate further understanding.
View Article and Find Full Text PDFMod Pathol
November 2024
Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa; Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland. Electronic address:
Case Rep Nephrol Dial
October 2024
Department of Renal Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
Introduction: Fibrillary glomerulonephritis (FGN) is a rare form of glomerular disease that accounts for less than 1 percent of all renal biopsies. It is characterized by pathognomonic electron microscopy findings of fibrillar deposits in the mesangium and glomerular capillary walls. FGN was initially considered to be an idiopathic disorder.
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