We report an unusual case of fibrillary glomerulonephritis (FGN) presenting as rapidly progressive renal failure and extensive crescent formation along with linear staining of capillary walls of the glomeruli on immunofluorescence, mimicking anti-glomerular basement membrane (anti-GBM) antibody-mediated disease. Laboratory results for circulating anti-GBM antibodies were negative. The subsequent electron microscopic findings were that of presence of electron-dense deposits in the glomerular mesangium and capillary walls, comprising of non-branching fibrils with an average diameter of 16 nm consistent with a diagnosis of FGN. This case illustrates the crucial role of electron microscopy in differential diagnosis of crescentic glomerulonephritis.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4421680 | PMC |
http://dx.doi.org/10.1093/ndtplus/sfr146 | DOI Listing |
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.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!