IgA vasculitis (Henoch-Schonlein purpura)

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.

Immunoglobulin A vasculitis (IgAV; formerly known as Henoch-Schonlein purpura [HSP]) is the most common vasculitis of childhood and presents with a purpuric rash, abdominal pain, arthritis/arthralgia, and glomerulonephritis.[1]Roberts PF, Waller TA, Brinker TM, et al. Henoch-Schonlein purpura: a review article. South Med J. 2007 Aug;100(8):821-4. http://www.ncbi.nlm.nih.gov/pubmed/17713309?tool=bestpractice.com

In 2012, the International Chapel Hill Consensus Conference Nomenclature of Vasculitides replaced the eponym Henoch-Schonlein purpura with IgAV to better reflect the pathophysiology.[2]Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11. https://www.doi.org/10.1002/art.37715 http://www.ncbi.nlm.nih.gov/pubmed/23045170?tool=bestpractice.com [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. https://www.doi.org/10.1007/s10157-013-0869-6 http://www.ncbi.nlm.nih.gov/pubmed/24072416?tool=bestpractice.com ​​​ However, the two terms are still sometimes used interchangeably in clinical practice. This topic focuses on the diagnosis and management of IgAV in children only. IgAV in adults is rare and beyond the scope of this topic.