Journal of Clinical Pediatrics >
Advances in pathogenesis, diagnosis and treatment of fibrillary glomerulonephritis
Received date: 2025-03-24
Accepted date: 2025-06-25
Online published: 2026-02-02
Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with unknown etiology. Member of DNAJ heat shock protein family B9 (DNAJB9) protein may be involved in its pathogenesis. The clinical manifestations of FGN are mostly nephrotic syndrome, which can be combined with hematuria, hypertension and renal insufficiency. A few patients show rapid progressive glomerulonephritis. It has important diagnostic value by electron microscopic examination of renal tissue combined with immunohistochemistry of DNAJB9 in FGN patients.Unfortunately, there is no specific therapy at present, and the prognosis is poor. In the future, it is urgent to explore the potential therapeutic targets of FGN to improve the quality of life of FGN patients. This article reviews the possible pathogenesis and clinical characteristics of FGN.
ZHANG Yuanyuan , ZHAO Dean . Advances in pathogenesis, diagnosis and treatment of fibrillary glomerulonephritis[J]. Journal of Clinical Pediatrics, 2026 , 44(2) : 167 -174 . DOI: 10.12372/jcp.2026.25e0295
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