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[Clinical and pathological analysis of fibrillary glomerulopathy]
1Institute of Nephrology, First Teaching Hospital, Beijing Medical University.
Abstract:
Four cases of fibrillary glomerulopathy (FGP) were reported for the first time in China. All patients had proteinuria, microscopic hematuria and hypertension. Renal function deteriorated in 2 cases. Pathological findings in light microscopy were sever: MPGN (2 cases), MN (1 case) and MsPGN (1 case). Congo-red stain of renal specimens was negative in all of them. Immunofluoscence (IF) in most of the cases (3/4 cases) showed granular IgG and C3 deposits in mesangium and glomerular basement membrane (GBM). Electron microscopy (EM) showed rich of fibrils distributed randomly over mesangium and GBM, the diameter of fibrils was 20.50 +/- 0.37nm. The diagnosis of FGP depends on EM examination. The actual incidence of FGP in China needs further studies.
Insights
Fibrillary glomerulopathy (FGP) is a rare kidney disease. Diagnosis relies on electron microscopy, showing characteristic fibrils, and requires further study in China.
Area of Science:
- Nephrology
- Pathology
- Electron Microscopy
Context:
- Fibrillary glomerulopathy (FGP) is an uncommon glomerular disease.
- This study reports the first four cases of FGP diagnosed in China.
Purpose:
- To describe the clinical, pathological, and ultrastructural features of FGP in Chinese patients.
- To highlight the diagnostic criteria and challenges for FGP.
Summary:
- Four Chinese patients presented with proteinuria, hematuria, and hypertension; two experienced renal function decline.
- Light microscopy showed various patterns (MPGN, MN, MsPGN), with negative Congo-red staining.
- Immunofluorescence revealed granular IgG and C3 deposits in three cases.
- Electron microscopy confirmed characteristic fibrils (20.50 ± 0.37 nm) in the mesangium and GBM, confirming FGP diagnosis.
Impact:
- Establishes the initial characterization of FGP in a Chinese cohort.
- Emphasizes the indispensable role of electron microscopy for definitive FGP diagnosis.
- Suggests a need for broader investigation into the prevalence of FGP in China.