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:[1][2][3][4][5][6][7][8][9][10]
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.
Plasmapheresis
- Plasmapheresis may be effective in delaying the progression of kidney disease and is usually done in addition to steroids.
References
- ↑ Schmoldt A, Benthe HF, Haberland G, Belaich A, Belaich JP, Prodanović Z, Obradović D, Petrović S, Nikolić L, Mutibarić A (September 1975). "Digitoxin metabolism by rat liver microsomes". Biochem. Pharmacol. 24 (17): 1639–41. PMID 10. Vancouver style error: initials (help)
- ↑ Augusto JF, Sayegh J, Delapierre L, Croue A, Tollis F, Cousin M, Subra JF (May 2012). "Addition of plasma exchange to glucocorticosteroids for the treatment of severe Henoch-Schönlein purpura in adults: a case series". Am. J. Kidney Dis. 59 (5): 663–9. doi:10.1053/j.ajkd.2011.12.015. PMID 22300649.
- ↑ Donghi D, Schanz U, Sahrbacher U, Recher M, Trüeb RM, Müllhaupt B, French LE, Hafner J (2009). "Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage". Dermatology (Basel). 219 (2): 167–70. doi:10.1159/000223237. PMID 19494483.
- ↑ Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM (February 2009). "Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review". Arch. Dis. Child. 94 (2): 132–7. doi:10.1136/adc.2008.141820. PMID 18701559.
- ↑ Saulsbury FT (March 2007). "Clinical update: Henoch-Schönlein purpura". Lancet. 369 (9566): 976–8. doi:10.1016/S0140-6736(07)60474-7. PMID 17382810.
- ↑ Share JB, Scherberger RR, Kaess H, Brückner S, Verbruggen R, Koivula T, Koivusalo M, Share JB (January 1976). "Review of drug treatment for Down's syndrome persons". Am J Ment Defic. 80 (4): 388–93. PMID 2011.
- ↑ Huber AM, King J, McLaine P, Klassen T, Pothos M (April 2004). "A randomized, placebo-controlled trial of prednisone in early Henoch Schönlein Purpura [ISRCTN85109383]". BMC Med. 2: 7. doi:10.1186/1741-7015-2-7. PMC 400510. PMID 15059282.
- ↑ Bogdanović R (December 2009). "Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment". Acta Paediatr. 98 (12): 1882–9. doi:10.1111/j.1651-2227.2009.01445.x. PMID 19650836.
- ↑ Alexander S (September 1988). "Patch testing and menstruation". Lancet. 2 (8613): 751. PMID 2901604.
- ↑ Frankle RT, Autrup H, Warwick GP, Bose KS, Sarma RH, Schomerus H, Buchta I, Arndt H, Anderson TR, Slotkin TA (January 1976). "Nutrition education in the medical school curriculum: a proposal for action: a curriculum design". Am. J. Clin. Nutr. 29 (1): 105–9. doi:10.1093/ajcn/29.1.105. Unknown parameter
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