Paediatrics

IgA Vasculitis

IgA immune-complex small-vessel vasculitis causes palpable purpura with variable joint, gastrointestinal and renal disease; the visible rash usually settles, but renal surveillance determines long-term safety.

Definition

IgA vasculitis, formerly Henoch-Schönlein purpura, is an IgA-dominant small-vessel vasculitis affecting skin, joints, gastrointestinal tract and kidneys.

First principles

One immune process explains four organ patterns

IgA vasculitis is an IgA1-dominant small-vessel vasculitis. Vessel inflammation and leakage produce palpable purpura in the skin, arthralgia or arthritis around joints, bowel oedema or bleeding, and glomerular inflammation with haematuria or proteinuria.

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