Nephrotic Syndrome

Primary glomerular diseases (grouped by pathology)

• Focal segmental glomerulosclerosis (40%)

idiopathic. HIV (collapsing variant), pamidronate. heroin (acute nephrotic syndrome) sustained hyperfiltration due to prior nephron loss, obesity, vesicoureteral reflux

• Membranous nephropathy (30%)

idiopathic, infection (especially HBV. also HCV. syphilis), autoimmune (especially SLE) carcinomas, drugs (gold, penicillamine, NSAIDs)

• Minimal change disease (20%. more common in children)

idiopathic. NSAIDs. Hodgkin's disease & other lymphoproliferative disorders

• Membranoproliferative GN (5%. mixed nephrotic nephritic features)

Type I: infection (especially HCV - cryos; IE. HBV. other chronic infxns). immune complex disease (SLE, cryos. Sjogren's), lymphoproliferative disorders, idiopathic Type II: very rare; autoAb blocks inactivation of C3 convertase - C3 nephritic factor

• Fibrillary-immunotactoid glomerulopathy (1%)

• Mesangial proliferative GN (likely atypical forms of MCD or FSGS, 5%)

Systemic diseases

• Diabetes mellitus: nodular glomerulosclerosis (Kimmelstiel-Wilson lesion); large kidneys hyperfiltration — microalbuminuria — dipstick • — nephrotic range (10-15 y) concomitant proliferative retinopathy seen in 90% of type 1 and 60% of type 2

• Amyloidosis: primary (Ig light chain) or secondary (serum amyloid)

• SLE: typically with membranous nephropathy (WHO class V)

• Cryoglobulinemia: typically with membranoproliferative GN

Workup (An!urn 2001:161:25)

• Urine sediment: usually benign w o concurrent nephritis: • oval fat bodies

• r o secondary causes t HbAic • retinopathy presumpt. dx of diabetic nephropathy unless another suggested ANA. anti-dsDNA. C3. C4. SPEP UPEP. fat pad bx. cryocrit, HBV. HCV. HIV. RPR

Treatment

• General: protein supplementation; diuretics for edema; treat hyperlipidemia

• ACEI/ARB: decrease proteinuria — slow nonimmunologic progression of renal disease

• Primary glomerular disease: steroids • cytotoxic therapy

• Secondary causes: treat underlying disease

Complications

• Malnutrition (protein loss)

• Thrombosis (especially renal vein, because loss of ATIII & other endogenous anticoags)

• Infection (especially encapsulated organisms, because loss of immunoglobulin)

• Accelerated atherosclerosis (hypercholesterolemia)

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