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.
- 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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History & Exam
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Definition
Epidemiology
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Citations
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]
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]
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]
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3. Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Clin Exp Nephrol. 2013 Oct;17(5):603-6.[Abstract][Full Text]
4. 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]
5. 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]
6. Nong BR, Huang YF, Chuang CM, et al. Fifteen-year experience of children with Henoch-Schönlein purpura in southern Taiwan, 1991-2005. J Microbiol Immunol Infect. 2007 Aug;40(4):371-6.[Abstract]
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8. Jauhola O, Ronkainen J, Koskimies O, et al. Clinical course of extrarenal symptoms in Henoch-Schonlein purpura: a 6-month prospective study. Arch Dis Child. 2010 Nov;95(11):871-6.[Abstract][Full Text]
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11. Leung AKC, Barankin B, Leong KF. Henoch-Schönlein purpura in children: an updated review. Curr Pediatr Rev. 2020;16(4):265-76. [Abstract]
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13. Tracy A, Subramanian A, Adderley NJ, et al. Cardiovascular, thromboembolic and renal outcomes in IgA vasculitis (Henoch-Schönlein purpura): a retrospective cohort study using routinely collected primary care data. Ann Rheum Dis. 2019 Feb;78(2):261-9.[Abstract][Full Text]
14. Hočevar A, Tomšič M, Rotar Ž. IgA vasculitis in adults: few certainties and many uncertainties. Ann Rheum Dis. 2020 Apr;79(4):e47.[Abstract][Full Text]
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19. 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;14(10):e0223218.[Abstract][Full Text]
20. Shin JI, Park JM, Shin YH, et al. Predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch-Schönlein purpura. Scand J Rheumatol. 2006 Jan-Feb;35(1):56-60.[Abstract]
21. 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]
22. Calvo-Río V, Hernández JL, Ortiz-Sanjuán F, et al. Relapses in patients with Henoch-Schönlein purpura: Analysis of 417 patients from a single center. Medicine (Baltimore). 2016 Jul;95(28):e4217.[Abstract][Full Text]
23. Fietta P. Systemic vasculitides: immunogenetics and familial clustering. Clin Exp Rheumatol. Mar-Apr 2004;22(2):238-51.[Abstract]
24. Ten Holder SM, Joy MS, Falk RJ, et al. Cutaneous and systemic manifestations of drug-induced vasculitis. Ann Pharmacother. 2002 Jan;36(1):130-47.[Abstract]
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26. Saulsbury FT. Henoch-Schonlein purpura. Curr Opin Rheumatol. 2001 Jan;13(1):35-40.[Abstract]
27. Kemper MJ. Primary IgA nephropathy and Henoch-Schonlein purpura nephritis. Monatsschr Kinderheilkd. 2004;152:257-64.
28. Chen O, Zhu XB, Ren P, et al. Henoch Schonlein purpura in children: clinical analysis of 120 cases. Afr Health Sci. 2013 Mar;13(1):94-9.[Abstract][Full Text]
29. Xu Y, Wang JJ, Liu FF, et al. Predisposing factors of childhood Henoch-Schönlein purpura in Anhui province, China. J Investig Med. 2019 Apr;67(4):771-8.[Abstract]
30. 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]
31. 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]
32. Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schönlein purpura. J Paediatr Child Health. 1998 Oct;34(5):405-9.[Abstract]
33. Simundic AM, Bölenius K, Cadamuro J, et al. Joint EFLM-COLABIOCLI recommendation for venous blood sampling. Clin Chem Lab Med. 2018;56(12):2015-38.[Abstract]
34. Brennan T; IAEM Guideline Development Committee. Henoch Schonlein purpura. March 2018 [internet publication].[Full Text]
35. Royal Children's Hospital Melbourne. Henoch-Schönlein purpura. January 2021 [internet publication].[Full Text]
36. Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, et al. Henoch-Schonlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. 1997 May;40(5):859-64.[Abstract]
37. Hahn D, Hodson EM, Willis NS, et al. Interventions for preventing and treating kidney disease in Henoch-Schönlein purpura (HSP). Cochrane Database Syst Rev. 2015 Aug 7;(8):CD005128.[Abstract][Full Text]
38. KDIGO clinical practice guidelines for glomerulonephritis. Chapter 11: Henoch-Schönlein purpura nephritis. Kidney Int Suppl (2011). 2012 Jun;2(2):218-20.[Abstract][Full Text]
39. 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]
40. 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]
41. Kanaan N, Mourad G, Thervet E, et al. Recurrence and graft loss after kidney transplantation for henoch-schonlein purpura nephritis: a multicenter analysis. Clin J Am Soc Nephrol. 2011 Jul;6(7):1768-72.[Abstract][Full Text]
42. Reamy BV, Servey JT, Williams PM. Henoch-Schönlein purpura (IgA vasculitis): rapid evidence review. Am Fam Physician. 2020 Aug 15;102(4):229-33.[Abstract]
43. Silva CA, Cocuzza M, Borba EF, et al. Cutting-edge issues in autoimmune orchitis. Clin Rev Allergy Immunol. 2012 Apr;42(2):256-63.[Abstract]
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45. Modi S, Mohan M, Jennings A. Acute scrotal swelling in Henoch-Schonlein purpura: case report and review of the literature. Urol Case Rep. 2016 May;6:9-11.[Abstract][Full Text]
46. Gohari A, Matsell DG, Mammen C, et al. Henoch-Schönlein purpura in children: Use of corticosteroids for prevention and treatment of renal disease. Can Fam Physician. 2020 Dec;66(12):895-7.[Abstract][Full Text]
47. Hernández-Rodríguez J, Carbonell C, Mirón-Canelo JA, et al. Rituximab treatment for IgA vasculitis: A systematic review. Autoimmun Rev. 2020 Apr;19(4):102490.[Abstract][Full Text]
48. ClinicalTrials.gov. Efficacy of colchicine to prevent skin relapses in adult's IgA vasculitis (COLCHIVAS). NCT04008316. November 2019 [internet publication].[Full Text]
49. Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child. 2005 Sep;90(9):916-20.[Abstract][Full Text]
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