Questions Under Study: We assessed the long-term follow up of all the patients with fibrillary glomerulonephritis diagnosed since 1992 at our centre of reference for renal pathology in Basel.
Methods: We performed a retrospective surveillance study with mail questionnaire based follow-up of all patients with the diagnosis of fibrillary glomerulonephritis found in the database of the department of renal pathology in Basel from 1992 to 2007. The outcome was assessed in terms of endstage renal disease (ESRD), death, reduction of proteinuria and improvement of estimated glomerular filtration rate (eGFR).
Results: We obtained sufficient follow up data from 16 out of 20 identified patients. The mean follow up time was 35 months (1-115.1). Six patients died (37.5%), three without having ESRD. Six patients (37.5%) reached ESRD, five of them went on hemodialysis. Thirteen patients (81.3%) received an immunosuppressive therapy with steroids, five of them in combination with cyclophosphamide. The group without immunosuppressive therapy was too small to compare the two groups. In relation to the histological pattern membranous glomerulonephritis (MGN) had a better outcome as compared to the other histological patterns.
Conclusions: FGN is a heterogeneous disease associated with significant risk of ESRD and mortality. The histological type of the glomerulonephritis may influence the course of the disease.
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http://dx.doi.org/10.4414/smw.2012.13578 | 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!