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.
Quick Reference
History & Exam
Key Factors
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Diagnostics Tests
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Definition
Epidemiology
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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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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]
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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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