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- Unnamed
- Sunday, May 13th, 2012 at 9:04:05pm MDT
- HSP
- o small-vessel leukocytoclastic vasculitis
- o tissue deposition of Ig-A containing immune complexes in affected organs
- o Classic tetrad
- o Rash
- o Abdominal pain
- o Arthritis/arthralgia
- o Glomerulonephritis
- o Unknown aetiology
- o Immune mediated
- o Infectious triggers proposed
- o Genetic factors implicated
- o Can be drug related
- o All of these correlations are unproven/unclear
- o Epidemiology
- o Mainly children
- o 3-15 yrs
- o 50% of cases < 5
- o Incidence in America 13.5 in 100, 000
- o More in whites
- o Males > females 2:1
- o History/Examination
- o Male, 3-15 yrs, prior URTI (more common in colder months)
- o Arthralgias
- Often assoc. with oedema
- Ankles/knees most common
- Present in ~80% of patients
- o Abdo pain
- In 50% pts. + nausea/vomiting
- o Rash
- Palpable purpure, typically non-blanching
- Can occur anywhere, usually in lower extremeties
- Occurs in ALL patients
- o Renal symptoms
- Half patients
- Haematuria/proteinuria/rbc casts
- o Uncommon historical features
- o Certain medications may be assoc.
- Antibiotics/phenytoin
- o 13% can have scrotal pain/swelling
- o Extremely rare
- Headache/seizures/pulmonary haemorrhage
- o Investigation
- o 1st tests
- Urinalyis
- • Rbcs/proteinuria/casts
- 24 hour urine for protein
- • Looking for renal involvement
- o Tests you can consider
- Serum IgA
- Serum Creatinine/EUC
- Coagulation studies (should be normal in HSP)
- Skin biopsy
- o DDx
- o ITP
- Low platelets in ITP, normal in HSP
- o Hypersensitivity vasculitis
- Usually doesn’t have renal involvement
- Skin biopsy to prove otherwise
- o Wegeners uncommon in kids
- o SLE uncommon in kids
- o RA
- Different age/different rash
- o Meningococcal septicaemia
- Abnormal coagulation / low platelet
- o Treatment (cortico are 1mg/kg/day for 3 months)
- o Depends on severity
- o Without nephritic-range proteinuria/GFR decline
- Just joint pain
- • Analgesics
- With severe oedema/scrotal involvement/abdo pain
- • Oral corticosteroids
- • Rest hydration elevation
- o Nephritic range protein uria
- Corticosteroids
- If rapidly progressive nephritis
- • Add immunosuppressants (cyclophosphamide for 12 weeks)
- Adjuncts
- • Plasmapheresis
- o Efficacy unknown as usually given with other treatments
- • Renal dialysis/transplant
- o Follow up
- o For at least 6 months with periodic urinalysis and BP monitoring
- o Usually excellent prognosis
- o Complete recovery in 94% kids and 89% adults
- o One third of patients have a recurrence within 4 months, but next episode is milder→
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