Wednesday, February 3, 2010


CASE 3: A 20 yo white male with one year of nephrotic syndrome, slow onset, and slowly rising creatinine to 2.0mg/dl and hematuria. A kidney biopsy done shows mesangial proliferation, EM with mesangial deposits, IF :3+ IgG, C1q,kappa and lambda, DIAGNOSIS?
Tip Variant Focal Segmental Glomeruloscelrosis
  0 (0%)
Membrano-proliferative GN
  1 (11%)

C1Q Nephropathy
  6 (66%)

Mesangio-proliferative GN
  1 (11%)

Light Chain Deposition Disease
  1 (11%)

The best option is C1Q Nephropathy.  This disease falls in the spectrum of Minimal Change and FSGS.
Classically, you see mesangial proliferation in most cases and the IF will be positive for just C1q, C3 and IgG.
EM will now show anything except mesangial deposits. It clinically can behave like any other immune complex disease.  Lupus has to be ruled out and other infectious causes leading to a concurrent immune complex disease have to be treated or ruled out.  The prognosis is usually not bad. From the Columbia series, 75% of them with C1Q nephropathy had stable renal function and 50% had complete or partial remission.  Only 2 patients progressed to ESRD. In other studies, the outcome was more like FSGS rather than MCD.
So they are more likely to progress to ESRD.  Most present with renal failure, nephrotic range proteinuria or hematuria. There are no treatment trials. Most people would treat it like MCD with 1mg/kg of Prednisone for 12 weeks and see what response they get. 

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