Diagnosis of IgA Vasculitis (Henoch-Schönlein Purpura) - French Vasculitis Study Group
1) Suggestive clinical manifestations
- Most common systemic vasculitis in childhood with an annual incidence of 3 to 26 per 100,000 children, occurring most frequently between 4 and 7 years
- Rare in adults, with an annual incidence of 0.1 to 1.8 per 100,000 individuals
- Clinical manifestations :
- Symmetric palpable purpura mainly in the pressure areas (may be necrotic or hemorrhagic)
- Arthralgias in 2/3 of patients
- Gastrointestinal involvement in 2/3 of patients : abdominal pain +++, potential severe complications
- Renal involvement occurs with a prevalence ranging from 45 to 85% : microscopic hematuria
- Elevated serum IgA levels in 50%
2) Presence of vasculitis with IgA1 -dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles)
- Dominant IgA deposits sometimes difficult to highlight, especially in the skin
- Systemic disease, skin-dominant variant or skin-limited (single-organ vasculitis)
- Leukocytoclastic IgA1 -dominant vasculitis of mostly post-capillary venules and also veins or arterioles in the skin, with vascular IgA deposits
Dr. Benjamin Terrier @TerrierBen
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