Related Experiment Videos
Fibrillary glomerulonephritis
B G Hsu1, C H Chang, S S Chiang
1Division of Nephrology, Department of Internal Medicine, Shin Kong Wu Ho-Su Memorial Hospital, 95, Wen-Chang Road, Shih-Lin, Taipei 111, Taiwan.
Abstract:
The occurrence of fibrillary glomerulonephritis is unusual in Taiwan, whereas it occurs in approximately 1% of renal biopsy specimens taken in the United States of American. This disease is characterized by extracellular randomly arranged non-branching Congo red-negative microfibrils within glomeruli. The microfibrils are less than 30 nm in diameter, and electron microscopy is essential for diagnosis. Differential diagnosis of the deposition of extracellular non-branching microfibrils within glomeruli is important because discrete diseases have different therapeutic and prognostic implications. The report will discuss two cases of biopsy-proved fibrillary glomerulonephritis who presented with proteinuria, hematuria, renal insufficiency, and hypertension. It is noteworthy that the renal function persistently went downhill, even though the physician treated the patients with corticosteroids, pulse treatment and immunosuppressive agents.
Insights
Fibrillary glomerulonephritis, a rare kidney disease, involves microfibril deposits. This condition showed poor response to standard treatments in two Taiwanese patients.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Fibrillary glomerulonephritis (FGN) is an uncommon glomerular disease characterized by Congo red-negative microfibril deposition.
- Diagnosis relies on electron microscopy due to the small size (<30 nm) and random arrangement of microfibrils.
- Distinguishing FGN from other microfibrillar diseases is crucial for appropriate management and prognosis.
Observation:
- Two cases of biopsy-proven FGN in Taiwan are presented.
- Patients exhibited typical symptoms including proteinuria, hematuria, renal insufficiency, and hypertension.
- The disease demonstrated a progressive decline in renal function.
Findings:
- Microfibrils in FGN are extracellular, randomly arranged, and non-branching.
- Electron microscopy is indispensable for identifying these characteristic ultrastructural findings.
- The pathological findings highlight the specific nature of microfibril accumulation in glomeruli.
Implications:
- FGN's rarity in Taiwan contrasts with its higher incidence in the United States.
- Treatment with corticosteroids, pulse therapy, and immunosuppressants proved ineffective in halting disease progression.
- These cases underscore the challenging nature of FGN and the need for further research into effective therapeutic strategies.