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fibrillary glomerulonephritis
Etiology: idiopathic
Epidemiology:
- rare, but more common than immunotactoid glomerulonephritis
- mean age 55-60 years
Pathology:
- deposition of randomly arranged fibrils derived from immunoglobulins that accumulate in the glomeruli
- fibrils larger than those associated with amyloidosis
- fibrils do not stain with Congo Red
Clinical manifestations:
- hypertension
- nephrotic syndrome
Laboratory:
- urinalysis
- urine protein generally in nephrotic range
- microscopic hematuria
Complications:
- 50% of cases progress to end-stage renal disease within 10 years of diagnosis
- may recur after renal transplantation
Management:
- no controlled trials
- plasmpheresis, prednisone, cytotoxic agenets, NSAIDs & colchicine
Related
immunotactoid glomerulonephritis
General
glomerulonephritis (GN, nephritic syndrome)
References
- Medical Knowledge Self Assessment Program (MKSAP) 15, 18.
American College of Physicians, Philadelphia 2009, 2018.