Histologic regression of fibrillary glomerulonephritis: the first report of biopsy-proven spontaneous resolution of disease
| Autor: | Samih H. Nasr, Joseph P. Grande, Miroslav Sekulic, Lynn D. Cornell |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
Pathology
medicine.medical_specialty medicine.medical_treatment kidney biopsy immunosuppressive therapy 030232 urology & nephrology 030204 cardiovascular system & hematology immune complex glomerulonephritis Diabetic nephropathy 03 medical and health sciences Rare Diseases 0302 clinical medicine Biopsy medicine Transplantation Proteinuria medicine.diagnostic_test business.industry diabetic nephropathy Fibrillary Glomerulonephritis Glomerulonephritis Immunosuppression medicine.disease Secondary Focal Segmental Glomerulosclerosis Nephrology fibrillary glomerulopathy medicine.symptom business Nephrotic syndrome |
| Zdroj: | Clinical Kidney Journal |
| ISSN: | 2048-8513 2048-8505 |
| DOI: | 10.1093/ckj/sfx045 |
| Popis: | Fibrillary glomerulonephritis (FGN) is a rare immune complex type glomerulonephritis characterized by glomerular deposition of randomly oriented fibrils measuring 10–30 nm in thickness, and typically presents with proteinuria with or without renal insufficiency and hematuria. We present a case in which a patient initially presented at age 41 years with nephrotic-range proteinuria and hypertension; a kidney biopsy showed FGN. The patient was treated with angiotensin receptor blockage only, without immunosuppression as per patient preference, and the level of protein in the urine improved. During the follow-up period of 17 years, the patient developed type 2 diabetes mellitus. The patient re-presented with nephrotic-range proteinuria 17 years later, at the age of 58 years. A kidney biopsy was performed and showed diffuse diabetic glomerulosclerosis with secondary focal segmental glomerulosclerosis and related vascular changes. There was no evidence of FGN by immunofluorescence or electron microscopy. Although FGN has been rarely reported to regress clinically, this is the first documented case of histologic regression of FGN. The potential for FGN fibrils to regress spontaneously is important in the management of FGN patients considering that currently available immunosuppressive agents have limited efficacy, and is an encouraging finding for future studies aiming to find a cure for the disease. |
| Databáze: | OpenAIRE |
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