Henoch-Schonlein Purpura (HSP)

IgA-mediated generalised vasculitis involving small vessels of the skin, GI tract, kidneys, joints, rarely the lungs and CNS

Aetiology

  • 75% of cases occur in children age 2-11, rare in infants
  • 75% of patients have had a preceding infection (URTI, pharyngeal, GI), most commonly GAS, 1-3 weeks before HSP develops

Clinical presentation

  • Purpuric rash typically over buttocks and lower limbs
  • Colicky abdominal pain
  • Bloody diarrhoea
  • Joint pain +/- swelling
  • Renal involvement (50%)
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Investigations

  • Clinical diagnosis but other tests may be useful if uncertainty
    • Bloods:
      • FBC, U+Es, LFTs, inflammatory markers, complement levels
      • ANCAs and specific antibodies
      • Connective tissue disease screen
    • Imaging - CXR and/or CT scan
    • Nerve conduction tests
    • Tissue biopsy for definative diagnosis in uncertain cases
  • Urine dipstick should always be performed to check for renal involvement

Management

  • Usually self-limiting within 8 weeks, may be relapses (months-years after resolution)
  • Urinalysis and BP should be closely monitored during course of illness due to risk of renal failure - may require nephrology referral