Fibrillary Glomerulonephritis: Current Insights into Pathology, Pathogenesis, and Clinical Outcomes.
- 1Department of Anatomical Pathology, Division of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
- 2Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
- 3College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
Abstract
Fibrillary glomerulonephritis (FGN) is a rare, predominantly glomerular disease characterized by non-amyloid fibrils measuring 10-30 nm in diameter, deposited in the glomeruli in a random, non-branching pattern. The diagnosis is supported by ultrastructural demonstration of randomly arranged fibrils, and DNAJB9 immunohistochemistry has improved diagnostic confidence in routine clinical practice. The recent discovery of DNAJB9 has significantly improved diagnostic accuracy through immunohistochemistry, reducing reliance on ultrastructural studies. FGN commonly presents with proteinuria, often in the nephrotic range, along with hematuria and varying degrees of renal dysfunction. Histologically, fibrillary glomerulonephritis exhibits a spectrum of patterns, including mesangioproliferative, membranoproliferative, diffuse proliferative, membranous, and diffuse sclerotic forms, with mesangioproliferative and membranoproliferative patterns being the most commonly encountered. Immunofluorescence typically reveals polyclonal IgG and C3 deposits. The disease is often idiopathic but may be associated with autoimmune conditions, infections, monoclonal gammopathy, or malignancy. FGN generally carries a poor prognosis, with many patients progressing to end-stage renal disease. This review summarizes recent advances in the understanding of FGN, including its pathology, pathogenesis, and clinical implications.
Related
- Case Report: Monotypic IgG1-λ fibrillary glomerulonephritis associated with rheumatoid arthritis.
- Clinicopathological Features of DLL3 Expression and T-Cell Infiltration in LCNEC: Insights From Pathology and Clinical Response to Tarlatamab.
- The role of vascular pathology in SMA: implications for pathogenesis and treatment.
- Treatment-refractory autoimmune glial fibrillary acidic protein astrocytopathy: Clinical features, predictive models, therapy
- Mechanistic insights into lactate-driven pathogenesis in diabetic kidney disease
BibTeX
@article{alsaad2026fibrillary,
title = {{Fibrillary Glomerulonephritis: Current Insights into Pathology, Pathogenesis, and Clinical Outcomes.}},
author = {Khaled O Alsaad and Abdulrahman K Alabdulsalam and Turki Al Hussain and Hadeel Al Mana},
journal = {Saudi journal of medicine \& medical sciences},
year = {2026},
month = oct,
doi = {10.4103/sjmms.sjmms_876_25},
url = {https://europepmc.org/article/MED/42974343},
note = {CC BY-NC-SA},
}
RIS
TY - JOUR
TI - Fibrillary Glomerulonephritis: Current Insights into Pathology, Pathogenesis, and Clinical Outcomes.
AU - Khaled O Alsaad
AU - Abdulrahman K Alabdulsalam
AU - Turki Al Hussain
AU - Hadeel Al Mana
PY - 2026
DA - 2026/10/14
JO - Saudi journal of medicine & medical sciences
DO - 10.4103/sjmms.sjmms_876_25
UR - https://europepmc.org/article/MED/42974343
AB - Fibrillary glomerulonephritis (FGN) is a rare, predominantly glomerular disease characterized by non-amyloid fibrils measuring 10-30 nm in diameter, deposited in the glomeruli in a random, non-branching pattern. The diagnosis is supported by ultrastructural demonstration of randomly arranged fibrils, and DNAJB9 immunohistochemistry has improved diagnostic confidence in routine clinical practice. The recent discovery of DNAJB9 has significantly improved diagnostic accuracy through immunohistochemistry, reducing reliance on ultrastructural studies. FGN commonly presents with proteinuria, often in the nephrotic range, along with hematuria and varying degrees of renal dysfunction. Histologically, fibrillary glomerulonephritis exhibits a spectrum of patterns, including mesangioproliferative, membranoproliferative, diffuse proliferative, membranous, and diffuse sclerotic forms, with mesangioproliferative and membranoproliferative patterns being the most commonly encountered. Immunofluorescence typically reveals polyclonal IgG and C3 deposits. The disease is often idiopathic but may be associated with autoimmune conditions, infections, monoclonal gammopathy, or malignancy. FGN generally carries a poor prognosis, with many patients progressing to end-stage renal disease. This review summarizes recent advances in the understanding of FGN, including its pathology, pathogenesis, and clinical implications.
SN - 1658-631X
SN - 2321-4856
ER -