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Clinical practice:

Diagnosis and management of Henoch–Schönlein purpura

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Abstract

Henoch–Schönlein purpura (HSP) is the most common vasculitis of childhood. In this review, the main clinical features and complications are described. Although most features are self-limiting, renal disease is the most likely to result in long-term morbidity. Treatment of HSP nephritis is controversial, and the evidence for both prevention and treatment of established disease is reviewed. Follow-up for children presenting with HSP should be for at least 6 months and should include regular urine testing for proteinuria and haematuria and a blood pressure measurement. Women with a history of HSP during childhood are at increased risk of complications (such as proteinuria and hypertension) during pregnancy and should be monitored closely. This paper describes current practice with regard to investigation and management and proposes a practical care pathway of the management of a child with HSP.

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References

  1. Aalberse J, Dolman K, Ramnath G et al (2007) Henoch Schönlein purpura in children: an epidemiological study among Dutch paediatricians on incidence and diagnostic criteria. Ann Rheum Dis 66(12):1648–1650

    Article  PubMed  Google Scholar 

  2. Allen AC, Willis FR, Beattie TJ, Feehally J (1998) Abnormal IgA glycosylation in Henoch–Schönlein purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant 13(4):930–934

    Article  CAS  PubMed  Google Scholar 

  3. Amoli MM, Calviño MC, Garcia-Porrua C et al (2004) Interleukin 1beta gene polymorphism association with severe renal manifestations and renal sequelae in Henoch–Schönlein purpura. J Rheumatol 31(2):295–298

    CAS  PubMed  Google Scholar 

  4. Calviño MC, Llorca J, García-Porrúa C et al (2001) Henoch Schonlein purpura in children from northwest Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 80(5):279–290

    Article  Google Scholar 

  5. Chang WL, Yang YH, Lin YT, Chiang BL (2004) Gastrointestinal manifestations in Henoch–Schönlein purpura: a review of 261 patients. Acta Paediatr 93(11):1427–1431

    Article  PubMed  Google Scholar 

  6. Chang WL, Yang YH, Wang LC et al (2005) Renal manifestations in Henoch–Schönlein purpura: a 10-year clinical study. Pediatr Nephrol 20(9):1269–1272

    Article  PubMed  Google Scholar 

  7. Chartapisak W, Opastiraku S, Willis NS et al (2009) Prevention and treatment of renal disease in Henoch–Schönlein purpura: a systematic review. Arch Dis Child 94(2):132–137

    Article  CAS  PubMed  Google Scholar 

  8. Coppo R, Peruzzi L, Amore A et al (2007) IgACE: a placebo-controlled, randomized trial of angiotensin-converting enzyme inhibitors in children and young people with IgA nephropathy and moderate proteinuria. J Am Soc Nephrol 18(6):1880–1888

    Article  CAS  PubMed  Google Scholar 

  9. Counahan R, Winterborn MH, White RH et al (1977) Prognosis of Henoch–Schönlein nephritis in children. Br Med J 2(6078):11–14

    Article  CAS  PubMed  Google Scholar 

  10. Dudley J, Afifi E, Gardner A et al (2000) Polymorphism of the ACE gene in Henoch–Schönlein purpura nephritis. Pediatr Nephrol 14(3):218–220

    Article  CAS  PubMed  Google Scholar 

  11. Dudley J, Smith G, Llewellyn-Edwards A, Tizard E (2007) Randomised placebo controlled trial to assess the role of early prednisolone on the development and progression of Henoch–Schönlein purpura nephritis. Pediatr Nephrol 22:1457 (abst 270)

    Google Scholar 

  12. Edström Halling S, Söderberg MP, Berg UB (2009) Treatment of severe Henoch–Schönlein and immunoglobulin A nephritis. A single center experience. Pediatr Nephrol 24(1):91–97

    Article  PubMed  Google Scholar 

  13. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of Henoch–Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360(9341):1197–1202

    Article  PubMed  Google Scholar 

  14. Goldstein AR, White RH, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch–Schönlein nephritis. Lancet 339(8788):280–282

    Article  CAS  PubMed  Google Scholar 

  15. Ha TS, Lee JS (2007) Scrotal involvement in childhood Henoch–Schönlein purpura. Acta Paediatr 96(4):552–555

    Article  PubMed  Google Scholar 

  16. Heberden W (1802) Commentaries on the history and cure of disease chapter 78: De purpueis maculis. Payne, London

    Google Scholar 

  17. Henoch EH (1889) Lectures on children’s diseases. A handbook for practitioners and students. New Sydenham Society, London

    Google Scholar 

  18. Huber AM, King J, McLaine P et al (2004) A randomized, placebo-controlled trial of prednisone in early Henoch Schönlein purpura. BMC Med 2:7

    Article  PubMed  Google Scholar 

  19. Lamireau T, Rebouissoux L, Hehunstre JP (2001) Intravenous immunoglobulin therapy for severe digestive manifestations of Henoch–Schönlein purpura. Acta Paediatr 90(9):1081–1082

    Article  CAS  PubMed  Google Scholar 

  20. Lewis M, Shaw J, Reid C et al (2006) Demography and management of childhood established renal failure in the UK. In UK Renal Registry. The Ninth Annual Report, pp 227–241

  21. Martin S, Cramer CH, Heikenen J, Gitomer JJ (2006) Gastrointestinal symptoms of Henoch–Schönlein purpura treated with mycophenolate mofetil. J Pediatr Gastroenterol Nutr 43(2):245–247

    Article  PubMed  Google Scholar 

  22. Mollica F, Li Volti S, Garozzo R, Russo G (1992) Effectiveness of early prednisone treatment in preventing the development of nephropathy in anaphylactoid purpura. Eur J Pediatr 151(2):140–144

    Article  CAS  PubMed  Google Scholar 

  23. Nadrous HF, Yu AC, Specks U, Ryu JH (2004) Pulmonary involvement in Henoch–Schönlein purpura. Mayo Clin Proc 79(9):1151–1157

    Article  PubMed  Google Scholar 

  24. Narchi H (2005) Risk of long term renal impairment and duration of follow up recommended for Henoch–Schönlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child 90(9):916–920

    Article  CAS  PubMed  Google Scholar 

  25. Ozaltin F, Bakkaloglu A, Ozen S et al (2004) The significance of IgA class of antineutrophil cytoplasmic antibodies (ANCA) in childhood Henoch–Schönlein purpura. Clin Rheumatol 23(5):426–429

    Article  PubMed  Google Scholar 

  26. Ozçakar ZB, Yalçinkaya F, Cakar N et al (2008) MEFV mutations modify the clinical presentation of Henoch–Schönlein purpura. J Rheumatol 35(12):2427–2429

    Article  PubMed  Google Scholar 

  27. Ozen S, Ruperto N, Dillon MJ et al (2006) EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 65(7):936–941

    Article  CAS  PubMed  Google Scholar 

  28. Ozkaya O, Söylemezoğlu O, Gönen S et al (2006) Renin-angiotensin system gene polymorphisms: association with susceptibility to Henoch–Schönlein purpura and renal involvement. Clin Rheumatol 25(6):861–865

    Article  PubMed  Google Scholar 

  29. Reinehr T, Bürk G, Andler W (2000) Does steroid treatment of abdominal pain prevent renal involvement in Henoch–Schönlein purpura? J Pediatr Gastroenterol Nutr 31(3):323–324

    Article  CAS  PubMed  Google Scholar 

  30. Rettig P, Cron RQ (2003) Methotrexate used as a steroid-sparing agent in non-renal chronic Henoch–Schönlein purpura. Clin Exp Rheumatol 21(6):767–769

    CAS  PubMed  Google Scholar 

  31. Ronkainen J, Ala-Houhala M, Antikainen M et al (2006) Cyclosporine A versus Methylprednisolone pulses in the treatment of severe Henoch–Schönlein Nephritis. Pediatr Nephrol 21(10):1531

    Google Scholar 

  32. Ronkainen J, Koskimies O, Ala-Houhala M et al (2006) Early prednisone therapy in Henoch–Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr 149(2):241–247

    Article  CAS  PubMed  Google Scholar 

  33. Ronkainen J, Nuutinen M, Koskimies O (2002) The adult kidney 24 years after childhood Henoch–Schönlein purpura: a retrospective cohort study. Lancet 360(9334):666–670

    Article  PubMed  Google Scholar 

  34. Rosenblum ND, Winter HS (1987) Steroid effects on the course of abdominal pain in children with Henoch–Schönlein purpura. Pediatrics 79(6):1018–1021

    CAS  PubMed  Google Scholar 

  35. Saulsbury FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78(6):395–409

    Article  CAS  Google Scholar 

  36. Siomou E, Serbis A, Salakos C et al (2008) Masked severe stenosing ureteritis: a rare complication of Henoch–Schönlein purpura. Pediatr Nephrol 23(5):821–825

    Article  PubMed  Google Scholar 

  37. Stewart M, Savage JM, Bell B, McCord B (1988) Long term renal prognosis of Henoch–Schönlein purpura in an unselected childhood population. Eur J Pediatr 147(2):113–115

    Article  CAS  PubMed  Google Scholar 

  38. Sunderkötter C, Bonsmann G, Sindrilaru A, Luger T (2005) Management of leukocytoclastic vasculitis. J Dermatolog Treat 16(4):193–206

    Article  PubMed  Google Scholar 

  39. Tarshish P, Bernstein J, Edelmann CM Jr (2004) Henoch–Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 19(1):51–56

    Article  PubMed  Google Scholar 

  40. Tizard EJ, Hamilton-Ayres MJJ (2008) Henoch Schönlein Purpura. Arch Dis Child Educ Pract Ed 93:1–8

    Article  CAS  PubMed  Google Scholar 

  41. Wang L, Huang FC, Ko SF, Cheng MT (2003) Successful treatment of mesenteric vasculitis caused by Henoch–Schönlein purpura with methylprednisolone pulse therapy. Clin Rheumatol 22(2):140–142

    Article  CAS  PubMed  Google Scholar 

  42. Wen YK, Yang Y, Chang CC (2005) Cerebral vasculitis and intracerebral hemorrhage in Henoch–Schönlein purpura treated with plasmapheresis. Pediatr Nephrol 20(2):223–225

    Article  PubMed  Google Scholar 

  43. Wortmann SB, Fiselier TJ, Van De Kar NC et al (2006) Refractory severe intestinal vasculitis due to Henoch-Schönlein purpura: successful treatment with plasmapheresis. Acta Paediatr 95(5):622–623

    Article  PubMed  Google Scholar 

  44. Zaffanello M, Fanos V (2009) Treatment-based literature of Henoch–Schönlein purpura nephritis in childhood. Pediatr Nephrol 24(10):1901–1911

    Article  PubMed  Google Scholar 

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The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Paediatric Nephrology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK

    Hugh J. McCarthy & E. Jane Tizard

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  1. Hugh J. McCarthy
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  2. E. Jane Tizard
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Correspondence to Hugh J. McCarthy.

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McCarthy, H.J., Tizard, E.J. Clinical practice:. Eur J Pediatr 169, 643–650 (2010). https://doi.org/10.1007/s00431-009-1101-2

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