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.

Long-term complications are rare but there is a risk of chronic kidney disease.

Early, accurate diagnosis is important to initiate appropriate management.

IgA vasculitis (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 immunoglobulin A vasculitis (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 used interchangeably in clinical practice.