Guideline for management of vasculitis syndrome (JCS 2008). Japanese Circulation Society.

Abstract

Vasculitis syndromes are classified, according to the size of the affected vessels, into large-vessel, medium-vessel, and small-vessel vasculitis (Table 1). Large-sized vessel vasculitis, ie, vasculitis occurring in the aorta and its major branches to the extremities, the head and the neck, includes Takayasu arteritis and temporal arteritis. Medium-sized vessel vasculitis, ie, vasculitis occurring in the major arteries and their branches to the visceral organs, includes polyarteritis nodosa (PAN), Kawasaki disease, and Buerger disease. Small-sized vessel vasculitis occurs in the arterioles, capillaries, and venules. In some cases, small arteries are also affected. Small-sized vessel vasculitis is classified according to the involvement of immune complexes. Vasculitis involving immune complexes include Henoch-Schönlein purpura, essential cryoglobulinemia, and malignant rheumatoid arthritis (MRA) (rheumatoid vasculitis). Small-sized vessel vasculitis not involving immune complexes include microscopic polyangiitis (MPA), Wegener’s granulomatosis, and allergic granulomatous angiitis. Since these three conditions are associated with the presence of marker antibodies, ie