Henoch-Schönlein purpura medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical therapy
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
- Cyclosporine
- Dipyridamole
- High-dose IV immunoglobulin G
- Danazol
- Fish oil
- Mycophenolate mofetil
- 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, mycophenolate mofetil and urokinase must be tested before their use is consistently advocated. Guidelines for prescribing azathioprine in dermatology have been established.
A randomized clinical trial of cyclosporine with methylprednisolone pulses in HSP with nephritis found that cyclosporine was superior and had many fewer complications. A study of 12 patients with severe HSP nephritis indicated that patients did well with methylprednisolone at 30 mg/kg/day for 3 days followed by oral corticosteroids at 2 mg/kg/day for 2 months, cyclophosphamide at 2 mg/kg/day for 2 months, and dipyridamole at 5 mg/kg/day for 6 months.
Some have noted that parvovirus B19–associated HSP must be recognized in adults because the treatment of choice is IV gamma globulin combined with anti-tumor necrosis factor (TNF)-α therapy. In contrast, immunosuppressive therapy may lead to a persistent or worsening disease course in these patients.