Henoch-Schönlein purpura medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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
- Corticosteroids are indicated in patients with
- 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.