IgA Vasculitis (Henoch-Schönlein Purpura) Flashcards
Define
HSP is an IgA mediated autoimmune vasculitis of childhood
- Most common vasculitis of childhood – affects the small vessels
- Boys 3-10y, peaks in winter, often preceded by URTI (2-3 days; otherwise if 1-12 weeks, think post-infectious)
- IgA and IgG complex and deposit in organs activating complement
Symptoms
Purpuric Rash (100%)
* Extensor surface of legs, arms, buttocks, ankles
* Urticarial -> maculopapular; spares trunk
Arthralgia and periarticular oedema (60-80%):
* Large Joints
* Joint pain and swelling of knees and ankles
Abdominal pain (60%)
* Can be treated with corticosteroids
* Colicky abdominal pain
* Haematemesis, melena, intussusception
* Orchitis
Glomerulonephritis (20-60% -> 97% within 3m of onset):
* Microscopic or macroscopic haematuria
* Nephrotic syndrome (rare)
Investigations
1st: FBC, clotting screen, urine dipstick, U&Es
Urinalysis: RBCs, proteinuria, casts, urea, creatinine, 24hr protein -> to rule out meningococcal sepsis
Increased IgA, normal coagulation
Follow-up (weekly for 1 month, 2-weekly for 2 months, 3 months, 6 months, 12 months):
* BP measurements
* Urine dipstick (haematuria)
Management
Most cases will resolve spontaneously within 4 weeks
Joint pain can be managed with paracetamol or ibuprofen
If there is scrotal involvement or severe oedema/ abdominal pain, PO prednisolone may be given
IV corticosteroids are recommended in patients with nephrotic-range proteinuria and those with declining renal function
Renal transplant may be considered in end-stage renal disease
Complications
Complications
Persistent haematuria or proteinuria is a risk factor for progressive CKD
Common complications: intussusception, acute renal impairment, arthritis, testicular pain, pancreatitis
< 1% progress to ESRF
Rare: MI, pulmonary haemorrhage, pleural effusion, GI bleeding
Prognosis
Recurrence can occur in up to 1/3 within 4-6 months of presentation
All children with HSP should be followed for a year
