Henoch-Schönlein purpura medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Medical therapy

Medical treatment of HSP:

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 in a dosage of 1 mg/kg/day for 2 weeks and then tapered over 2 more weeks may shorten the duration of abdominal pain and joint symptoms.
• In patients with a contraindication to steroids are given factor-VIII for abdominal pain.

A review of randomized clinical trials for any intervention used to improve renal disease in children with HSP noted that data were very limited except for short-term prednisone; moreover, prednisone had no benefit in preventing serious long-term renal disease.

Treatment of overt HSP includes methylprednisolone pulse therapy and prednisone and other immunosuppressive medications.

If prednisone is used, a regimen consisting of 1-2 mg/kg/day PO for 7 days is recommended.

Antihypertensives may be indicated with renal involvement.

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 IVIg infusion has been reported.
• Refractory chronic HSP can be treated with Rituximab.

• Azathioprine has been used to treat skin symptoms.