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Highlights & Basics
- IgA vasculitis (formerly known as Henoch-Schonlein purpura) is the most common vasculitis of childhood.
- Rash of palpable purpura is present in all cases.
- Most cases are self-limiting or resolve with symptomatic treatment. Immunosuppressive therapy is indicated for nephritis, orchitis, and other organ- or life-threatening manifestations.
- Long-term complications are rare but there is a risk of chronic kidney disease.
- Early, accurate diagnosis is important to initiate appropriate management.
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Definition
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Citations
Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis - the SHARE initiative. Rheumatology (Oxford). 2019 Sep 1;58(9):1607-16.[Abstract][Full Text]
UK Kidney Association. The initial management of IgA vasculitis (Henoch Schönlein Purpura) in children and young people. Dec 2022 [internet publication].[Full Text]
UK Kidney Association. The management of complications associated with IgA vasculitis (Henoch Schönlein Purpura) in children and young people. Dec 2022 [internet publication].[Full Text]
Hahn D, Hodson EM, Craig JC. Interventions for preventing and treating kidney disease in IgA vasculitis. Cochrane Database Syst Rev. 2023 Feb 28;(2):CD005128.[Abstract][Full Text]
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4S):S1-276.[Full Text]
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17. Eisenstein EM, Navon-Elkan P. Acute rheumatic fever associated with Henoch-Schonlein Purpura: report of three cases and review of the literature. Acta Paediatr. 2002;91(11):1265-7.[Abstract]
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26. Wei CC, Lin CL, Shen TC, et al. Atopic dermatitis and association of risk for Henoch-Schönlein purpura (IgA vasculitis) and renal involvement among children: results from a population-based cohort study in Taiwan. Medicine (Baltimore). 2016 Jan;95(3):e2586.[Abstract][Full Text]
27. Shi D, Chan H, Yang X, et al. Risk factors associated with IgA vasculitis with nephritis (Henoch-Schönlein purpura nephritis) progressing to unfavorable outcomes: a meta-analysis. PLoS One. 2019 Oct 1;14(10):e0223218.[Abstract][Full Text]
28. Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schonlein purpura. Arthritis Rheum. 1990 Aug;33(8):1114-21.[Abstract]
29. Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis - the SHARE initiative. Rheumatology (Oxford). 2019 Sep 1;58(9):1607-16.[Abstract][Full Text]
30. Ozen S, Pistorio A, Iusan SM, et al; Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis. 2010 May;69(5):798-806.[Abstract][Full Text]
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34. Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schönlein purpura. J Paediatr Child Health. 1998 Oct;34(5):405-9.[Abstract]
35. Royal Children's Hospital Melbourne. Henoch-Schönlein purpura. January 2021 [internet publication].[Full Text]
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38. Hahn D, Hodson EM, Craig JC. Interventions for preventing and treating kidney disease in IgA vasculitis. Cochrane Database Syst Rev. 2023 Feb 28;(2):CD005128.[Abstract][Full Text]
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41. Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch purpura nephritis. Pediatr Nephrol. 1998 Apr;12(3):238-43.[Abstract]
42. Ma Y, Han F, Chen L, et al. The impact of intravenous methylprednisolone pulses on renal survival in anti-neutrophil cytoplasmic antibody associated vasculitis with severe renal injury patients: a retrospective study. BMC Nephrol. 2017 Dec 29;18(1):381.[Abstract][Full Text]
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49. Williams CEC, Lamond M, Marro J, et al. A narrative review of potential drug treatments for nephritis in children with IgA vasculitis (HSP). Clin Rheumatol. 2023 Dec;42(12):3189-200.[Abstract][Full Text]
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51. Saulsbury FT. Henoch-Schonlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999 Nov;78(6):395-409.[Abstract]
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