Henoch-Schönlein purpura medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Henoch-Schönlein Purpura (HSP) is usually treated with supportive management by adequate hydration, balancing fluid and electrolyte, and controlling hypertension. Symptoms such as arthritis, edema, fever are treated with acetaminophen, leg elevation, and adequate hydration. Pharmacological management includes use of analgesics, NSAIDs, corticosteriods and various other immuno-suppressants. Plasmapheresis may be effective in delaying the progression of kidney disease and is usually done in addition to steroids.
Medical therapy
Medical treatment of HSP:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]
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 dose- 1 mg/kg/day for 14 days and then tapered over another 14 days help relieve the joint and abdominal symptoms.
- In patients with a contraindication to steroids are given factor-VIII for abdominal pain.
- Overt HSP
- Methylprednisolone pulse therapy
- Prednisone
- Other immunosuppressive medications.
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
- 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 IV Ig infusion has been reported.
- Refractory chronic HSP can be treated with Rituximab.
- Azathioprine has been used to treat skin symptoms.
- 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)
- ↑ Bluman J, Goldman RD (November 2014). "Henoch-Schönlein purpura in children: limited benefit of corticosteroids". Can Fam Physician. 60 (11): 1007–10. PMC 4229160. PMID 25551129.
- ↑ 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
|pmida=ignored (help) - ↑ Saulsbury FT (February 1993). "Corticosteroid therapy does not prevent nephritis in Henoch-Schönlein purpura". Pediatr. Nephrol. 7 (1): 69–71. PMID 8280195.
- ↑ Lu Z, Song J, Mao J, Xia Y, Wang C (May 2017). "Evaluation of Mycophenolate Mofetil and Low-Dose Steroid Combined Therapy in Moderately Severe Henoch-Schönlein Purpura Nephritis". Med. Sci. Monit. 23: 2333–2339. PMC 5444683. PMID 28515415.
- ↑ Chen O, Zhu XB, Ren P, Wang YB, Sun RP, Wei DE (March 2013). "Henoch Schonlein Purpura in children: clinical analysis of 120 cases". Afr Health Sci. 13 (1): 94–9. doi:10.4314/ahs.v13i1.26. PMC 3645106. PMID 23658574.
- ↑ Du Y, Hou L, Zhao C, Han M, Wu Y (May 2012). "Treatment of children with Henoch-Schönlein purpura nephritis with mycophenolate mofetil". Pediatr. Nephrol. 27 (5): 765–71. doi:10.1007/s00467-011-2057-9. PMID 22081165.
- ↑ Hahn D, Hodson EM, Willis NS, Craig JC (August 2015). "Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP)". Cochrane Database Syst Rev (8): CD005128. doi:10.1002/14651858.CD005128.pub3. PMID 26258874.