Henoch-Schönlein purpura medical therapy: Difference between revisions
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*Management of HSP is primarily supportive and includes | *Management of HSP is primarily supportive and includes | ||
**Adequate hydration | **Adequate hydration | ||
**Monitoring renal complications by balancing fluid and electrolyte, and controlling | **Monitoring renal complications by balancing fluid and electrolyte, and controlling hypertension. | ||
*Symptoms such as arthritis, edema, fever are treated | *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. | |||
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 (IV) methylprednisolone daily for 3-7 days plus cyclophosphamide 100-200 mg/d administered orally (PO) | |||
*'''Maintenance''' | |||
*Prednisone 100-200 mg PO every other day plus cyclophosphamide 100-200 mg/d PO 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 6 months by abruptly discontinuing cyclophosphamide and tapering prednisone completely | |||
Other agents | |||
Azathioprine | |||
Cyclophosphamide | |||
Cyclosporine | |||
Dipyridamole | |||
High-dose IV immunoglobulin G (IVIg) | |||
Danazol | |||
Fish oil | |||
Of these, only cyclophosphamide has been shown to be effective in a randomized controlled trial. Although some studies have reported success, cyclosporine does not have sufficient clinical data to establish its utility in this setting. [67] | |||
Azathioprine, mycophenolate mofetil and urokinase must be tested before their use is consistently advocated. Guidelines for prescribing azathioprine in dermatology have been established. [68] No convincing studies have yet been conducted regarding the use of IVIg, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP. [69] | |||
A randomized clinical trial of cyclosporine with methylprednisolone pulses in HSP with nephritis found that cyclosporine was superior and had many fewer complications. [70] 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. [71] | |||
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. | |||
Massive GI hemorrhage in isolated intestinal HSP that is responsive to IVIg infusion has been reported. [72] IVIg was used in a complex case of HSP with brain hemorrhage, but more work must be done to validate the use of this treatment. [73] | |||
Dapsone has been used to treat associated purpuras and arthralgias. Iqbal and Evans found it to be effective for HSP. [74] | |||
Rituximab has been noted to be a successful treatment for severe refractory chronic HSP. [75] | |||
Factor VIII concentrate has been used to relieve abdominal pain when corticosteroids are contraindicated. Recombinant factor VIIa has been used with very pronounced factor XIII deficiency and compartment syndrome. | |||
Treatment of complicated HSP with mycophenolate mofetil has been reported in a series of patients. | |||
Revision as of 17:06, 7 April 2018
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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.
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 (IV) methylprednisolone daily for 3-7 days plus cyclophosphamide 100-200 mg/d administered orally (PO)
- Maintenance
- Prednisone 100-200 mg PO every other day plus cyclophosphamide 100-200 mg/d PO 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 6 months by abruptly discontinuing cyclophosphamide and tapering prednisone completely
Other agents
Azathioprine Cyclophosphamide Cyclosporine Dipyridamole High-dose IV immunoglobulin G (IVIg) Danazol Fish oil Of these, only cyclophosphamide has been shown to be effective in a randomized controlled trial. Although some studies have reported success, cyclosporine does not have sufficient clinical data to establish its utility in this setting. [67]
Azathioprine, mycophenolate mofetil and urokinase must be tested before their use is consistently advocated. Guidelines for prescribing azathioprine in dermatology have been established. [68] No convincing studies have yet been conducted regarding the use of IVIg, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP. [69]
A randomized clinical trial of cyclosporine with methylprednisolone pulses in HSP with nephritis found that cyclosporine was superior and had many fewer complications. [70] 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. [71]
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.
Massive GI hemorrhage in isolated intestinal HSP that is responsive to IVIg infusion has been reported. [72] IVIg was used in a complex case of HSP with brain hemorrhage, but more work must be done to validate the use of this treatment. [73]
Dapsone has been used to treat associated purpuras and arthralgias. Iqbal and Evans found it to be effective for HSP. [74]
Rituximab has been noted to be a successful treatment for severe refractory chronic HSP. [75]
Factor VIII concentrate has been used to relieve abdominal pain when corticosteroids are contraindicated. Recombinant factor VIIa has been used with very pronounced factor XIII deficiency and compartment syndrome.
Treatment of complicated HSP with mycophenolate mofetil has been reported in a series of patients.