Table 1.

Classification of the CNS vasculitides according to the affected vessel size and neurological manifestations.

Large vessel	Takayasu arteritis Giant cell arteritis
Medium vessel	Polyarteritis nodosa Kawasaki disease
Small vessel	ANCA-associated: Microscopic polyangiitis Granulomatosis with polyangiitis (Wegener‘s granulomatosis) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) Immune complex vasculitis: Henoch-Schoenlein purpura
Variable vessel	Behçet’s disease
Vasculitis associated with systemic disease	Lupus vasculitis Rheumatoid vasculitis
Single organ vasculitis	Primary angiitis of the CNS Inflammatory cerebral amyloid angiopathy

Modified from the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides [8]. ANCA—antineutrophil cytoplasmic antibodies; CNS—central nervous system.