Nephrotic syndrome

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Definition

  • Syndrome characterized by:
    • Significant proteinuria > 3g/day
    • Hyperlipidemia/lipiduria
    • Anasarca
    • Hypoalbuminemia <35g/L

Differential Diagnosis

  • Non-proliferative glomerulonephritis
    • Minimal change disease
      • Primary
      • Drugs
        • NSAIDs and COX-2 inhibitors
        • Ampicillin, rifampicin, cephalosporins
        • Lithium
        • D-penicillamine and tiopronin
        • Pamidronate
        • Sulfasalazine and 5-ASAs
        • Interferon-gamma
      • Neosplasms
        • Hodgkin lymphoma
        • Non-Hodgkin lymphoma
        • Leukemia
      • Lupus nephritis V (membranous nephritis)
    • Focal segmental glomerulosclerosis
      • Primary
      • Severe obesity and other hyperfiltration syndromes
      • Infections
        • HIV
        • Parvovirus B19
        • CMV
        • EBV
      • Drugs
        • Heroin
        • Interferons
        • Pamidronate
        • Anabolic steroids
        • mTOR inhibitos
        • Calcineurin inhibitos
        • Anthracyclines
        • Lithium
      • Other
        • Sickle cell anemia
        • Thrombotic microangiopathy
        • Genetic disorders
    • Membranous nephropathy
      • Primary
      • Lupus
      • Drugs
        • Gold
        • Penicillamine
        • NSAIDs
      • Infections
        • Hepatitis B and C
        • Malaria
        • Syphilis
      • Neoplasms
        • Non-Hodgkin lymphoma
        • Solid cancers
      • IgG4 disease

Most common causes

  • Membranous nephropathy (24 percent)
  • Minimal change disease (16 percent)
  • Lupus (14 percent)
  • FSGS (12 percent): more common in black patients
  • Amyloidosis (6 to 17 percent): more common in older patients
  • Membranoproliferative glomerulonephritis (7 percent)
  • IgA nephropathy (6 percent)

Epidemiology

  • If < 20 years old
    • Minimal change (70%)
    • Membranous (20%)
    • Focal segmental glomerulosclerosis (10%)
  • If > 20 years old
    • Membranous (70%)
    • Minimal change (20%)
    • Focal segmental glomerulosclerosis (10%)

Management

  • If < 20 years old
    • Treat empirically with steroids, as the majority are primary minimal change disease
  • If > 20 years old
    • Needs kidney biopsy to direct treatment