Introduction
Vasculitis is inflammation of blood vessel walls, which narrows, blocks or weakens them (aneurysms) and damages the organs they supply. In children the two big ones are IgA vasculitis (Henoch–Schönlein purpura) and Kawasaki disease; the rest are rare. They are classified by the size of vessel involved (EULAR/PRINTO/PRES).
IgA vasculitis (Henoch–Schönlein purpura)
The commonest vasculitis of childhood, mostly at 3–10 years, often 1–3 weeks after an upper respiratory infection. IgA immune complexes deposit in small vessels of skin, gut, joints and kidneys.
Diagnosis (Ankara 2008)
Palpable purpura (mandatory), mainly on the legs and buttocks, with at least one of:
- Abdominal pain (colicky; bleeding; intussusception, often ileo-ileal).
- Arthritis or arthralgia (knees, ankles; no lasting damage).
- Kidney involvement: haematuria and/or proteinuria.
- Biopsy showing IgA deposits.
Platelets and clotting are normal: the purpura is from vasculitis, not low platelets. Scrotal swelling (orchitis) can mimic torsion.
Management and outlook
- Most settle in 4 weeks: rest, paracetamol or NSAIDs (if kidneys are normal); one third have a recurrence.
- Steroids for severe abdominal pain or orchitis (they do not prevent kidney disease).
- Kidney follow-up: urine dipstick and blood pressure regularly for at least 6–12 months. Nephritis (nephrotic range proteinuria, raised creatinine) needs biopsy and immunosuppression; it is the only part that can cause long-term harm (chronic kidney disease in 1–2%).