Snapshot A 67-year-old man with a history of type 2 diabetes and hypertension presents with worsening pedal edema. His diabetes has been poorly controlled and his last HbA1C was measured at 9.7 about 3 months ago. He reports no other symptoms. Physical exam reveals bilateral 2+ pitting edema to the knees bilaterally, and fundoscopy reveals cotton wool spots. A urinalysis shows 3+ protein, and serum albumin is 2.5. He is prescribed an ACE-inhibitor by his physician. (Diabetic Nephropathy) Introduction Secondary nephrotic syndrome is a multisystem disease Primary nephrotic syndrome refers to disease limited to kidney Presentation Hypoalbuminemia (serum albumin of <3.0 g/dl) Proteinuria > 3.5 g/day Generalized edema (anasarca) caused by loss of albumin / decreased oncotic pressure Hyperlipidemia with lipiduria low albumin stimulates liver to produce cholesterol Hypercoaguable state Differential Nephrotic Syndrome Nephritic Syndrome Chronic Disease Proteinuria + + + + / - + / - Hematuria + / - + + + + / - Cells + / - + RBCs / + WBCs + / - Casts Fatty Casts RBC and granular casts Waxy/pigmented/granular casts Lipids Free fat dropletsOval fat bodies Evaluation Labs show hypoalbuminemia (serum albumin of <3.0 g/dL) proteinuria > 3.5 g/day hyperlipidemia with lipiduria low albumin stimulates liver to produce cholesterol Definitive diagnosis made by renal biopsy Generalized Treatment Management of primary disease process causing nephrotic syndrome Protein restriction Salt restriction Diuretic therapy for edema HMG-CoA reductase inhibitor for hyperlipidemia Diabetic Glomerulonephropathies Most common cause of end stage renal disease in USA Early manifestation is microalbuminuria Biopsy shows pathognomnic Kimmelstiel-Wilson nodules Treatment can slow progression with ace inhibitors strict glycemic and hypertension control transplant is only treatment after disease has progressed HIV Glomerulonephropathies Usually seen in HIV acquired through IV drug use Presents with focal segmental glomerulonephritis Treat HIV Lupus Glomerulonephritis Type I no renal involvement Type II mesangial disease with focal segmental glomerular pattern Type III focal proliferative disease treat with aggressive prednisone +/- cyclophosphamide Type IV most severe form diffuse proliferative disease presents as combination of nephritic and nephrotic disease classic wire loop abnormality (image above) treat with prednisone, cyclophosphamide, transplant Type V membranous disease indistinguishable from other primary membraneous glomerulonephropathies Treatment: consider prednisone