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what type of vasculitis is Henoch Schonlein purpura?
multisystem small vessel vasculitis
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which organs does HSP affect?
skin, joints, GI, kidney
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what is HSP also known as?
anaphylactoid purpura
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what age does HSP affect?
2-11 years old
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is HSP more in boys or girls?
- boys (even though autoimmune!)
- 2:1
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what is the cause of HSP?
- exact unknown
- immunopathological involvement
- increased IgA in serum
- make complexes, activate complement and deposited in affected organs
- precipitate an inflammatory response with vascultiis
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which other disease does HSP have an overlap with?
IgA nephropathy
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what time of year is HSP more frequent in?
winter
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what are the trigger agents for HSP?
- infectious agents - URTI
- drugs
- food
- insect bites
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what are the clinical features of HSP?
- purpuric rash - but initially urticarial. affecting predominantly extensors (buttocks, legs) symmetrical
- arthritis with pain and swelling - knees and ankles
- cramping abdominal pain, GI bleed - complication intussusception ileoileal
- renal: haematuria, proteinuria in a minority of patients. can be severe. clinical glomerulonephritis or nephrotic syndrome
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differential diagnosis of HSP
- other cause of abdominal pain
- other causes of purpuric or petechial pain eg ITP
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how is diagnosis of HSP made?
clinical
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what are the useful Ix for HSP?
- FBC - normal platelet count rules out ITP
- renal funciton U&E
- US abdo - rule out intussecption
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what is Rx of HSP?
- symptomatic and supportive
- paracetamol or NSAIDs control pain
- oral steroids occasional used if severe abdominal pain but ensure intussesception not present
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what is prognosis of HSP
majority full recovery in 4-6 weeks
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what does long term prognosis of HSP depend on?
presence and severity of NEPHRITIS
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why do you have to follow up HSP for prolonged period?
to monitor renal function
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what % of pts with HSP progress to end stage renal failure
only 1% pts progress to end stage renal disease
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