Vasculitis is currently classified by the size of vessels involved by inflammation: large-vessel vasculitis such as Takayasu arteritis and giant cell arteritis, medium-sizedvessel vasculitis such as polyarteritis nodosa and Kawasaki disease, and small-vessel vasculitis such as immune complex small-vessel vasculitis and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. 1 ANCA-associated vasculitis is characterized by necrotizing small vessels, including capillaries, venules, arterioles, and sometimes small arteries, together with the presence of autoantibodies against neutrophils, myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or both. ANCA-associated vasculitis has three categories, microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, previously called Wegener granulomatosis), and eosinophilic granulomatosis with polyangiitis (EGPA, previously called Churg-Strauss syndrome).