Henoch-Schonlein Purpura (IgA Vasculitis)

5 year old presents with acute onset diffuse/colicky abdominal pain, arthralgias/arthritis, purpura. Symptoms preceded by sore throat. Hypertension and palpable, non-blanching purpura primarily located on lower extremities.

Note: Vasculitis-associated rashes are non-blanching

Note: Vasculitis-associated rashes are non-blanching

  • Labs

    • CBC may show thrombocytopenia

    • Obtain initial CMP to evaluate creatinine, serum albumin

    • Evaluate early morning CMP for creatinine > 30 µmol/L

    • Obtain PT/PTT/INR

    • Evaluate urinalysis for proteinuria, hematuria

    • Consider skin biopsy

  • Severe abdominal pain: Consider ultrasound to rule out intussusception

  • Treatment

    • Continue supportive therapy

    • Arthritic pain: Acetaminophen per pediatric dosing

    • Positive strep test and age > 3 years and GFR > 30: Amoxicillin 50 mg/kg qd x 10 days (maximum daily dose 1,000 mg/day)

    • Renal involvement as evidenced by elevated creatinine, proteinuria, and/or hematuria

      • Prednisone 1 mg/kg/day x 2 weeks followed by 2 week taper

      • HTN: Amlodipine 0.1 mg/kg/day (maximum daily dose 5 mg/day)

      • Consult nephrology

      • Monitor BP, creatinine, and urinalysis monthly for six months

    • Refractory abdominal/arthritic pain and/or scrotal swelling: Consider prednisone (see dosing above)

  • Counseling

    • Parents informed that 95% of cases resolve spontaneously within 8 weeks

    • Avoid ibuprofen due to abdominal pain and concern for renal involvement

    • Relapses can occur for up to 10 years following illness

Notes

  • Epidemiology

    • Most common systemic vasculitis of childhood

      • Typically occurs between age 3-15 years with peak prevalence at age 4-6 years

      • Only 10% of cases occur in adults

    • Sometimes associated with preceding streptococcal pharyngitis

  • Pediatric Rheumatology European Society Criteria

    • Mandatory: Purpura or petechiae with lower limb predominance

    • At least one of the following:

      • Arthritis or arthralgia

      • Acute onset diffuse abdominal pain

      • Renal involvement: Proteinuria or hematuria

      • Histopathology: Leukoclastic vasculitis or proliferative glomerulonephritis with IGA deposits

  • Treatment

    • Calcium channel blockers (e.g. amlodipine) are indicated for hypertension associated with renal involvement

    • Corticosteroids

      • Prophylactic treatment does not prevent renal disease (SOR A)

      • Early treatment may reduce joint and abdominal pain