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

More general terms

• glomerulonephritis (GN, nephritic syndrome)

Additional terms

• immunotactoid glomerulonephritis