HSP, ITP

Question 1

Henoch-Schonlein Purpura

Answer 1

IgA-mediated autoimmune hypersensitivity vasculitis of childhood

Question 2

clinical features HSP

Answer 2

purpura
arthritis
abdo pain
GI bleed
orchitis (inflammation of testes - 1 or both)
nephritis

Question 3

epidemiology HSP

Answer 3

6-20 per 100,000
90% of cases occur in childhood
peak prevalence aged 4-6yo
rare in infants and young children
m:f 1.5-2:1
caucasians most at risk
mostly in winter months

Question 4

presentation HSP

Answer 4

50-90% have preceding URTI
mildly ill, low grade fever
symmetrical, erythematous macular rash, esp back of legs, buttocks and ulnar side of arms
within 24h, macules develop into purpura, which may coalesce to resemble bruises
typically purpura slightly raised and palpable
abdo pain and diarrhoea may precede rash
may also cause n&v
joint pain esp in knees and ankles
joints may be swollen, but no permanent deformity
renal involvement in 40%, but rarely proceeds to end stage renal failure

Question 5

DDx HSP

Answer 5

rule out intussusception
connective tissue disease eg SLE
other causes of purpuric rash eg thrombocytopenia
other causes of glomerulonephritis
acute haemorrhagic oedema of infancy

Question 6

management HSP

Answer 6

usually self limiting

treatment primarily supportive

Question 7

complications HSP

Answer 7

renal involvement
rare:
MI
pulmonary haemorrhage
pleural effusion
intussusception
GI bleed
bowel infarct
seizure
mononeuropathies

Question 8

immune thrombocytopenia - ITP

Answer 8

autoimmune disorder with reduced number of circulating platelets
due to increased platelet destruction, and sometimes due to reduced production

Question 9

presentation ITP

Answer 9

may be asymptomatic
life threatening intracranial haemorrhage
most common is petechiae or bruising
up to 25% epistaxis