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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