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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

(A) GRANULOMATOUS

• Wegener’s granulomatosis

• Churg-Strauss syndrome

(B) NON-GRANULOMATOUS

• 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