Henoch-Schönlein purpura medical therapy

Henoch-Schönlein Purpura (HSP) is usually treated with supportive management by adequate hydration, balancing fluid and electrolyte, and controlling hypertension. Symptoms such as arthritis, edema, fever are treated with acetaminophen, leg elevation, and adequate hydration. Pharmacological management includes use of analgesics, NSAIDs, corticosteriods and various other immuno-suppressants. Plasmapheresis may be effective in delaying the progression of kidney disease and is usually done in addition to steroids.

Medical treatment of HSP:^{[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]}

Supportive Management

• Management of HSP is primarily supportive and includes
  • Adequate hydration
  • Monitoring renal complications by balancing fluid and electrolyte, and controlling hypertension.
• Symptoms such as arthritis, edema, fever are treated with acetaminophen, leg elevation, and adequate hydration.

Pharmacological Management

• Analgesics
• NSAIDs (Nonsteroidal anti-inflammatory drug) and acetaminophen reduces the joint pain and are effective against purpura. NSAIDs are used with caution in patients with renal insufficiency.
• Corticosteroids
  • Corticosteroids are indicated in patients with
    • Subcutaneous edema such as Severe soft tissue edema, severe scrotal edema
    • Nephritis
    • Arthralgia
    • Abdominal GI dysfunction

• Prednisone dose– 1 mg/kg/day for 14 days and then tapered over another 14 days help relieve the joint and abdominal symptoms.
• In patients with a contraindication to steroids are given factor-VIII for abdominal pain.
• Overt HSP
  • Methylprednisolone pulse therapy
  • Prednisone
  • Other immunosuppressive medications.

Fredda’s treatment protocols in patients with severe HSP:

• Induction

• 250-750 mg of intravenous Methylprednisolone daily for 3-7 days plus Cyclophosphamide 100-200 mg/d administered orally.

• Maintenance
• Prednisone 100-200 mg orally every other day plus Cyclophosphamide 100-200 mg/day orally 30-75 days.

• Tapering
• Tapering off prednisone by approximately 25 mg/month (with the cyclophosphamide dose remaining constant)

• Discontinue
• Discontinuance of treatment after at least six months by abruptly discontinuing cyclophosphamide and tapering prednisone completely

• Other agents
  • Azathioprine
  • Cyclophosphamide
  • Mycophenolate mofetil
  • Cyclosporine
  • Dipyridamole
  • Urokinase
  • High-dose IV immunoglobulin G
  • Danazol
  • Fish oil

• Cyclophosphamide has been effective of all the above.
• Dapsone has been used to treat associated purpuras and arthralgias.
• Isolated intestinal HSP with massive GI bleed is responsive to IV Ig infusion has been reported.
• Refractory chronic HSP can be treated with Rituximab.

• Azathioprine has been used to treat skin symptoms.

Plasmapheresis

• Plasmapheresis may be effective in delaying the progression of kidney disease and is usually done in addition to steroids.  

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