UK Kidney Association guideline review: ‘The initial management of IgA vasculitis (Henoch-Schönlein purpura) in children and young people’ in conjunction with ‘The management of complications-associated IgA vasculitis (Henoch-Schönlein purpura) in children and young people’

Abstract

IgA vasculitis (IgAV), previously known as Henoch-Schönlein purpura (HSP), is an IgA-mediated autoimmune vasculitis typically affecting the skin, joints, gastrointestinal (GI) tract and kidneys. In IgAV, IgA complexes are deposited on the endothelium of the small vessels in these organs, causing inflammation. IgAV has an incidence of approximately 1 in 5000, predominantly affects children between the ages of 2 and 10 years and peaks between 4 and 7 years,1 can affect teenagers and adults, and is a common childhood condition seen by paediatricians, general practitioners (GPs) and emergency department (ED) doctors across the UK. Despite this, the research in this field is still limited, so currently, there are discrepancies in both the initial management and management of complications related to IgAV. While most cases of IgAV self-resolve with conservative management or management in primary and secondary care, some children will develop severe, life-changing or life-threatening complications such as acute GI bleeding and end-stage renal disease requiring tertiary centre involvement.2 It is important that careful specialist referral, screening and early management of these children occur to ensure that these complications are prevented or treated in a timely manner. ### Information about the current guideline The current guidelines were published in December 2022 by the UK Kidney Association (UKKA), and are the first national guidelines for the initial management of IgAV and the management of complications associated with IgAV in children and young people. In 2019, the first international guidelines providing recommendations for diagnosis and treatment were published by SHARE (Single Hub and Access Point for paediatric Rheumatology in Europe) with the same aim to provide evidence-based recommendations and to standardise treatments throughout countries and centres.3 Until now, due to lack of best practice recommendations in the UK, experts in different National Health Service trusts have been formulating guidelines based on the most recent research. …