1. 65 year old female with a h/o familial Mediterranean fever, diabetes, proteinuria (2.7 g), hematuria (20-30rbc’s) – no rbc casts on urinanalysis. Labs normal including Cr, Complement, ANA, ANCA, MPO, PR3. Bad sinus infection in spring (3-4 weeks) with no PIGN signs. Renal bx: focal segmental crescentic and necrotizing gn (+/- 4 glomeruli with capillary necrosis; one cellular crescent), with focal mild mesangial proliferation. EM: IgG (2+), IgA (focal 1+), kappa+, lambda+, C3 trace, C1q neg - subepithelial/paramesangial deposits, No definite humps, No fibrils. No amyloid. Congo-red neg. Deposits not numerous. GBM variably thin and thick (range 137 – 537; 15% less than 200). DD: ANCA negative focal crescentic and necrotizing gn with IgG deposits Resolving PIGN (C3 trace). Plus: early diabetic nephropathy (thickening of GBM) superimposed on TBDM?? No association found between familial Mediterranean fever and TBMD

4. IgG Kappa IgG Lambda