Fibrillary-Immunotactoid Glomerulopathy - Rare
Cause Irreversible Renal Failure
Zharfbin A.1, Hušek K.2, Sobotová D.1
1II. interní klinika Lékařské fakulty MU a FN u sv. Anny, Brno, přednosta doc. MUDr. Jiří Zicha, CSc.2I. patologickoanatomický ústav Lékařské fakulty MU a FN u sv. Anny, Brno,přednosta prof. MUDr. Aleš Rejthar, CSc. |
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Summary:
Fibrillary-immunotactoid glomerulopathy (FITGP) is a rare cause of nephrotic syndrome. In this
patient, male 65-years-old, respectively, fibrillary glomerulonefritis (FGN) was diagnosed by percutaneous
renal biopsy. Clinically, the disease manifested with long term proteinuria and nephrotic
syndrome without any associated systemic disorder. Histologically, glomeruli showed
deposition of PAS+, fuchsinophilic and Orange G+ material in the mesangium and basal membranes.
Strong granular immunofluorescent IgG, C3, kappa and lambda light chains deposition was
present in the mesangium. Electronmicroscopically, depositions of fibrillary material in the expanded
mesangium and in the peripheral basal membranes were found. Randomly distributed
nonbranching fibrils measured 18 - 28 nm. After 18 months of follow-up, the therapy with corticosteroids
and Cylosporin A was without effect, and the disease progressed into chronic renal failure,
and after 24 months of biopsy the patient is undergoing hemodialysis now.
Key words:
Fibrillary glomerulonephritis - Fibrillar glomerulopathy-immunotactoid glomerulopathy
- Electronmicroscopy
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