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==Overview==
==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 therapy==
Medical treatment of HSP:<ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Belaich A, Belaich JP, Prodanović Z, Obradović D, Petrović S, Nikolić Lj, Mutibarić A |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |doi= |url=}}</ref><ref name="pmid25551129">{{cite journal |vauthors=Bluman J, Goldman RD |title=Henoch-Schönlein purpura in children: limited benefit of corticosteroids |journal=Can Fam Physician |volume=60 |issue=11 |pages=1007–10 |date=November 2014 |pmid=25551129 |pmc=4229160 |doi= |url=}}</ref><ref name="pmid22300649">{{cite journal |vauthors=Augusto JF, Sayegh J, Delapierre L, Croue A, Tollis F, Cousin M, Subra JF |title=Addition of plasma exchange to glucocorticosteroids for the treatment of severe Henoch-Schönlein purpura in adults: a case series |journal=Am. J. Kidney Dis. |volume=59 |issue=5 |pages=663–9 |date=May 2012 |pmid=22300649 |doi=10.1053/j.ajkd.2011.12.015 |url=}}</ref><ref name="pmid19494483">{{cite journal |vauthors=Donghi D, Schanz U, Sahrbacher U, Recher M, Trüeb RM, Müllhaupt B, French LE, Hafner J |title=Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage |journal=Dermatology (Basel) |volume=219 |issue=2 |pages=167–70 |date=2009 |pmid=19494483 |doi=10.1159/000223237 |url=}}</ref><ref name="pmid18701559">{{cite journal |vauthors=Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM |title=Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review |journal=Arch. Dis. Child. |volume=94 |issue=2 |pages=132–7 |date=February 2009 |pmid=18701559 |doi=10.1136/adc.2008.141820 |url=}}</ref><ref name="pmid17382810">{{cite journal |vauthors=Saulsbury FT |title=Clinical update: Henoch-Schönlein purpura |journal=Lancet |volume=369 |issue=9566 |pages=976–8 |date=March 2007 |pmid=17382810 |doi=10.1016/S0140-6736(07)60474-7 |url=}}</ref><ref name="pmid2011">{{cite journal |vauthors=Share JB, Scherberger RR, Kaess H, Brückner S, Verbruggen R, Koivula T, Koivusalo M, Share JB |title=Review of drug treatment for Down's syndrome persons |journal=Am J Ment Defic |volume=80 |issue=4 |pages=388–93 |date=January 1976 |pmid=2011 |doi= |url=}}</ref><ref name="pmid15059282">{{cite journal |vauthors=Huber AM, King J, McLaine P, Klassen T, Pothos M |title=A randomized, placebo-controlled trial of prednisone in early Henoch Schönlein Purpura [ISRCTN85109383] |journal=BMC Med |volume=2 |issue= |pages=7 |date=April 2004 |pmid=15059282 |pmc=400510 |doi=10.1186/1741-7015-2-7 |url=}}</ref><ref name="pmid19650836">{{cite journal |vauthors=Bogdanović R |title=Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment |journal=Acta Paediatr. |volume=98 |issue=12 |pages=1882–9 |date=December 2009 |pmid=19650836 |doi=10.1111/j.1651-2227.2009.01445.x |url=}}</ref><ref name="pmid2901604">{{cite journal |vauthors=Alexander S |title=Patch testing and menstruation |journal=Lancet |volume=2 |issue=8613 |pages=751 |date=September 1988 |pmid=2901604 |doi= |url=}}</ref><ref name="pmid2006">{{cite journal |vauthors=Frankle RT, Autrup H, Warwick GP, Bose KS, Sarma RH, Schomerus H, Buchta I, Arndt H, Anderson TR, Slotkin TA |title=Nutrition education in the medical school curriculum: a proposal for action: a curriculum design |journal=Am. J. Clin. Nutr. |volume=29 |issue=1 |pages=105–9 |date=January 1976 |pmida=2006 |doi=10.1093/ajcn/29.1.105 |url=}}</ref><ref name="pmid8280195">{{cite journal |vauthors=Saulsbury FT |title=Corticosteroid therapy does not prevent nephritis in Henoch-Schönlein purpura |journal=Pediatr. Nephrol. |volume=7 |issue=1 |pages=69–71 |date=February 1993 |pmid=8280195 |doi= |url=}}</ref><ref name="pmid28515415">{{cite journal |vauthors=Lu Z, Song J, Mao J, Xia Y, Wang C |title=Evaluation of Mycophenolate Mofetil and Low-Dose Steroid Combined Therapy in Moderately Severe Henoch-Schönlein Purpura Nephritis |journal=Med. Sci. Monit. |volume=23 |issue= |pages=2333–2339 |date=May 2017 |pmid=28515415 |pmc=5444683 |doi= |url=}}</ref><ref name="pmid23658574">{{cite journal |vauthors=Chen O, Zhu XB, Ren P, Wang YB, Sun RP, Wei DE |title=Henoch Schonlein Purpura in children: clinical analysis of 120 cases |journal=Afr Health Sci |volume=13 |issue=1 |pages=94–9 |date=March 2013 |pmid=23658574 |pmc=3645106 |doi=10.4314/ahs.v13i1.26 |url=}}</ref><ref name="pmid22081165">{{cite journal |vauthors=Du Y, Hou L, Zhao C, Han M, Wu Y |title=Treatment of children with Henoch-Schönlein purpura nephritis with mycophenolate mofetil |journal=Pediatr. Nephrol. |volume=27 |issue=5 |pages=765–71 |date=May 2012 |pmid=22081165 |doi=10.1007/s00467-011-2057-9 |url=}}</ref><ref name="pmid26258874">{{cite journal |vauthors=Hahn D, Hodson EM, Willis NS, Craig JC |title=Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP) |journal=Cochrane Database Syst Rev |volume= |issue=8 |pages=CD005128 |date=August 2015 |pmid=26258874 |doi=10.1002/14651858.CD005128.pub3 |url=}}</ref>
'''Supportive Management'''
'''Supportive Management'''
*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 hypertension.  
**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.   
*Symptoms such as [[arthritis]], edema, fever are treated with [[acetaminophen]], leg elevation, and adequate hydration.   


'''Pharmacological Management'''
'''Pharmacological Management'''
*'''''Analgesics'''''
*'''''[[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.
*'''''[[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]]'''''
**Corticosteroids are indicated in patients with
**[[Corticosteroids]] are indicated in patients with
***Subcutaneous edema such as Severe soft tissue edema, severe scrotal edema
***[[Subcutaneous]] edema such as Severe [[soft tissue]] edema, severe [[scrotal edema]]
***Nephritis
***[[Nephritis]]
***Arthralgia
***[[Arthralgia]]
***Abdominal GI dysfunction
***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.
* '''''[[Prednisone]]''''' '''dose'''- 1 mg/kg/day for 14 days and then tapered over another 14 days help relieve the joint and abdominal 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.
* In patients with a contraindication to [[steroids]] are given factor-VIII for abdominal pain.
 
* '''Overt HSP'''
Treatment of overt HSP includes methylprednisolone pulse therapy and prednisone and other immunosuppressive medications.  
** [[Methylprednisolone]] pulse therapy
 
** [[Prednisone]]
If prednisone is used, a regimen consisting of 1-2 mg/kg/day PO for 7 days is recommended.
** Other immunosuppressive medications.
 
'''Fredda's treatment protocols in patients with severe HSP:'''  
Antihypertensives may be indicated with renal involvement.
 
'''Fredda's treatment protocols in patients with severe HSP:'''


*'''Induction'''  
*'''Induction'''  


* 250-750 mg of intravenous Methylprednisolone daily for 3-7 days plus Cyclophosphamide 100-200 mg/d administered orally.
* 250-750 mg of intravenous [[Methylprednisolone]] daily for 3-7 days plus [[Cyclophosphamide]] 100-200 mg/d administered orally.


*'''Maintenance'''
*'''Maintenance'''
*Prednisone 100-200 mg orally every other day plus Cyclophosphamide 100-200 mg/day orally 30-75 days.
*[[Prednisone]] 100-200 mg orally every other day plus [[Cyclophosphamide]] 100-200 mg/day orally 30-75 days.


*'''Tapering'''
*'''Tapering'''
*Tapering off prednisone by approximately 25 mg/month (with the cyclophosphamide dose remaining constant)
*Tapering off [[prednisone]] by approximately 25 mg/month (with the [[cyclophosphamide]] dose remaining constant)


*'''Discontinue'''
*'''Discontinue'''
*Discontinuance of treatment after at least six months by abruptly discontinuing cyclophosphamide and tapering prednisone completely
*Discontinuance of treatment after at least six months by abruptly discontinuing [[cyclophosphamide]] and tapering [[prednisone]] completely


* '''Other agents'''
* '''Other agents'''
** Azathioprine
** ''[[Azathioprine]]''
** Cyclophosphamide
** ''[[Cyclophosphamide]]''
** Cyclosporine
** [[Mycophenolate sodium|Mycophenolate mofetil]]
** Dipyridamole
** ''[[Cyclosporine]]''
** High-dose IV immunoglobulin G  
** ''[[Dipyridamole]]''
** Danazol
** [[Urokinase]]
** Fish oil
** ''High-dose IV [[immunoglobulin G]]''
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]
** ''[[Danazol]]''
 
** ''[[Fish oil]]''
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]
* [[Cyclophosphamide]] has been effective of all the above.
* [[Dapsone adverse reactions|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.


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.
* [[Azathioprine]] has been used to treat skin symptoms.  


Treatment of complicated HSP with mycophenolate mofetil has been reported in a series of patients.  
'''[[Plasmapheresis]]'''
* [[Plasmapheresis]] may be effective in delaying the progression of kidney disease and is usually done in addition to [[steroids]].<sup>  </sup>


==References==
==References==

Latest revision as of 20:41, 15 April 2018

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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
  • Symptoms such as arthritis, edema, fever are treated with acetaminophen, leg elevation, and adequate hydration.

Pharmacological Management

  • 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

Fredda's treatment protocols in patients with severe HSP:

  • Induction
  • Discontinue
  • Discontinuance of treatment after at least six months by abruptly discontinuing cyclophosphamide and tapering prednisone completely
  • 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.

Plasmapheresis

  • Plasmapheresis may be effective in delaying the progression of kidney disease and is usually done in addition to steroids.  

References

  1. ↑ 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)
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ Saulsbury FT (March 2007). "Clinical update: Henoch-Schönlein purpura". Lancet. 369 (9566): 976–8. doi:10.1016/S0140-6736(07)60474-7. PMID 17382810.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ Alexander S (September 1988). "Patch testing and menstruation". Lancet. 2 (8613): 751. PMID 2901604.
  11. ↑ 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)
  12. ↑ Saulsbury FT (February 1993). "Corticosteroid therapy does not prevent nephritis in Henoch-Schönlein purpura". Pediatr. Nephrol. 7 (1): 69–71. PMID 8280195.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.

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