Immunoglobulin A Vasculitis (previously Henoch Schönlein Purpura (HSP))
Description Diagnostic criteria General and supportive measures Medicine treatment Referral ICD-10 code: D69.0 DESCRIPTION Immunoglobulin A vasculitis is an acute leucocytoclastic vasculitis of small blood vessels usually involving skin, gastrointestinal tract, joints and the kidney. Aetiology is unknown. Complications include: acute severe abdominal pain, bowel infarction/perforation
Description
Diagnostic criteria
General and supportive measures
Medicine treatment
Referral
ICD-10 code: D69.0
DESCRIPTION
Immunoglobulin A vasculitis is an acute leucocytoclastic vasculitis of small blood vessels usually involving skin, gastrointestinal tract, joints and the kidney. Aetiology is unknown.
Complications include:
- acute severe abdominal pain, bowel infarction/perforation;
- nephritis with renal impairment or nephrotic syndrome.
DIAGNOSTIC CRITERIA
Clinical
Syndrome consisting of:
- Non-thrombocytopenic palpable purpuric skin rash with a very typical distribution on lower extremities and buttocks. The rash occurs in 100% of cases, but may not be present initially. Trunk and upper extremities may be involved. Angiooedema of scalp, eyelids, lips and ears.
- Arthralgia/arthritis (60–70%): mostly of large joints, i.e. knees and ankles.
- Abdominal pain with ‘colic’ (60–70%): may develop gastrointestinal bleeding or intussusception or infarction.
- Renal involvement (25–50%) manifesting with haematuria and/or proteinuria.
Investigations
- No specific diagnostic test.
- Full blood count (FBC): Platelets may be normal or increased (differentiating this form of purpura from that caused by thrombocytopenia), mild leucocytosis is seen in some children. Normochromic anaemia often related to gastro-intestinal loss.
- Coagulation studies are normal.
- Urine test strip to evaluate renal involvement.
- Serum urea, creatinine, electrolytes and albumin with renal involvement.
- Check stools for occult or frank bleeding.
GENERAL AND SUPPORTIVE MEASURES
- Short period of immobilisation during acute arthritis.
- Soft diet for acute gastrointestinal involvement.
- Clinical review with blood pressure monitoring and urine test strip testing weekly or biweekly for first 2 months, then monthly for the next year.
MEDICINE TREATMENT
For arthritis, oedema, fever, malaise:
- Paracetamol, oral, 15 mg/kg/dose, 6 hourly as required.
OR
- Ibuprofen, oral, 10 mg/kg/dose 6 hourly.
- Reduce dosing interval to 8 hourly once pain starts improving.
For complicated HSP (severe pain, severe extrarenal symptoms or renal disease):
- Prednisone, oral, 1–2 mg/kg/dose once daily for 10 days in the morning.
- Reduce dose gradually over 2 weeks.
REFERRAL
HSP with complications, i.e. in patients with:
- Persistent proteinuria, persistent microscopic haematuria, hypertension or worsening renal function for age (renal biopsy indicated).
- Persistent abdominal pain.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Hospital Level, Paediatrics, 2023 Edition
Chapter 12: Rheumatology And Vasculitides, 12.1 Immunoglobulin A Vasculitis (previously Henoch Schönlein Purpura (HSP))