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European Journal of Pediatrics

Published in:

01-06-2010 | Review

Clinical practice:

Diagnosis and management of Henoch–Schönlein purpura

Authors: Hugh J. McCarthy, E. Jane Tizard

Published in: European Journal of Pediatrics | Issue 6/2010

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.

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):1648–1650CrossRefPubMed

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):930–934CrossRefPubMed

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):295–298PubMed

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):279–290CrossRef

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

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

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):132–137CrossRefPubMed

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):1880–1888CrossRefPubMed

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

10.

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

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)

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):91–97CrossRefPubMed

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):1197–1202CrossRefPubMed

14.

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

15.

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

16.

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

17.

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

18.

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

19.

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

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):245–247CrossRefPubMed

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):140–144CrossRefPubMed

23.

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

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):916–920CrossRefPubMed

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):426–429CrossRefPubMed

26.

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

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):936–941CrossRefPubMed

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):861–865CrossRefPubMed

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):323–324CrossRefPubMed

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):767–769PubMed

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

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):241–247CrossRefPubMed

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):666–670CrossRefPubMed

34.

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

35.

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

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):821–825CrossRefPubMed

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):113–115CrossRefPubMed

38.

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

39.

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

40.

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

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):140–142CrossRefPubMed

42.

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

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):622–623CrossRefPubMed

44.

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

Title: Clinical practice:
Diagnosis and management of Henoch–Schönlein purpura
Authors: Hugh J. McCarthy
E. Jane Tizard
Publication date: 01-06-2010
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 6/2010
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-009-1101-2

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Clinical practice:

Diagnosis and management of Henoch–Schönlein purpura

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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