A case of mistaken identity: fibrillary glomerulonephritis masquerading as crescentic anti-glomerular basement membrane disease
| Autor: | Mercury Lin, Arthur E. Anderson, Jimmy A Thomas, Dmitri Vasin, Charles E. Alpers |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
Lung Diseases
Pathology medicine.medical_specialty Anti-Glomerular Basement Membrane Disease Biopsy medicine.medical_treatment Kidney Glomerulus 030232 urology & nephrology Hemorrhage 030204 cardiovascular system & hematology urologic and male genital diseases Diagnosis Differential 03 medical and health sciences Glomerulonephritis 0302 clinical medicine medicine Humans Rapidly progressive glomerulonephritis Dialysis Aged Autoantibodies Basement membrane medicine.diagnostic_test urogenital system business.industry Fibrillary Glomerulonephritis Complement C3 General Medicine medicine.disease Capillaries Microscopy Electron medicine.anatomical_structure Microscopy Fluorescence Nephrology Immunoglobulin G Disease Progression Female business Follow-Up Studies Kidney disease |
| Zdroj: | Clinical Nephrology. 85:114-120 |
| ISSN: | 0301-0430 |
| DOI: | 10.5414/cn108667 |
| Popis: | Fibrillary glomerulonephritis (FGN) is a rare cause of rapidly progressive glomerulonephritis (RPGN). We report a case of FGN in which the patient presented with a clinical pulmonary-renal syndrome and whose kidney biopsy showed > 90% crescents on light microscopy. Immunofluorescence microscopy showed pseudo-linear IgG and C3 staining of the glomerular capillary walls resulting in an initial diagnosis of crescentic glomerulonephritis of anti-glomerular basement membrane (anti-GBM) antibody etiology. Electron microscopy showed fibrillary deposits permeating the glomerular capillary walls, characteristic of FGN. Although dialysis dependent at presentation and anuric at discharge, the patient recovered adequate renal function and urine output to come off dialysis at 20 weeks. A follow up biopsy performed at this stage showed progression of the underlying chronic kidney disease. This is the third reported case of FGN with a clinical presentation and histologic and immunofluorescence microscopic findings that closely mimicked anti-GBM antibody mediated disease. These cases demonstrate that FGN is a rare but important consideration in the differential diagnosis of RPGN. |
| Databáze: | OpenAIRE |
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