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Table 1 Classification of childhood-onset vasculitis based on the EULAR/PReS endorsed consensus criteria [6]

From: ANCA-associated vasculitis in childhood: recent advances

I Predominantly large-vessel vasculitis
• Takayasu arteritis
II Predominantly medium-sized vessel vasculitis
• Childhood polyarteritis nodosa
• Cutaneous polyarteritis
• Kawasaki disease
III Predominantly small-vessel vasculitis
• Wegener’s granulomatosis
• Churg-Strauss syndrome
• Microscopic polyangiitis
• Henoch-Schönlein purpura
• Isolated cutaneous leucocytoclastic vasculitis
• Hypocomplementic urticarial vasculitis
IV Other vasculitides
• Behçet disease
• Vasculitis secondary to infection (including hepatitis B associated polyarteritis nodosa), malignancies, and drugs, including hypersensivity vasculitis
• Vasculitis associated with connective tissue diseases
• Isolated vasculitis of the central nervous system
• Cogan syndrome
• Unclassified