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A. Examples

  1. ANCA Associated Disease
    1. Polyarteritis Nodosa (PAN)
    2. Wegener's Granulomatosis
  2. Hypersensitivity Vasculitis (Angiitis)
    1. Henoch-Schnlein Purpura (HSP)
    2. Serum Sickness
    3. Drug Reaction
    4. Hypersensitive Reaction to Infection
  3. Cryoglobulinemia
  4. Systemic Lupus Erythematosus (SLE)
  5. Takayasu Arteritis
  6. Giant Cell Arteritis (rarely)

B. Symptoms

  1. Renal Infarction
  2. Nephritis with azotemia
  3. Nephrotic Syndrome
  4. Renovascular Hypertension

C. Treatment [2]

  1. High dose iv methylprednisolone (eg. 1gm/day x 3 days)
  2. Cyclophosphamide - 1gm iv q month x 6-30 months
  3. Consider Azathioprine or Cyclosporine - 2.5-10mg/kg/day
  4. Cryoglobulinemia
    1. Severe cases respond to initial plasmapheresis + chemotherapy
    2. Interferon alpha can be effective in hepatitis C associated disease

References

  1. Ambrus JL and Sridhar NR. 1997. JAMA. 278(22):1938 abstract
  2. Glassock RJ. 1994. Cleve Clin J Med. 61(5):363 abstract