Diagnostic features:
(1) predominance of C1q deposits in mesangium and/or glomerular capillary wall on immunofluorescence
(2) electron dense deposits on electron microscopy
Cliinical presentation may include:
(1) nephrotic syndrome
(2) SLE
(3) asymptomatic hematuria
(4) asymptomatic proteinuria
(5) hypertension
(6) chronic renal failure
Histologic patterns in renal biopsies may include:
(1) minimal change disease
(2) focal segmental glomerulonephritis
(3) mesangial proliferative glomerulonephritis
Significance:
(1) Children with minimal change histology and the nephrotic syndrome may have frequent relapses and may show poor response to steroids but may respond to calcineurin inhibitor or mycophenolate mofetil.
(2) The presence of focal segmental GN and nephrotic syndrome may progress to chronic renal failure.
