Guideline for Management of Vasculitis Syndrome (JCS 2008)

Vasculitis syndromes are classified, according to the size of the affected vessels, into large-vessel, medium-vessel, and small-vessel vasculitis (Table 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 polyarteri-tis 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-Schnlein 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, *Described in the present guidelines. The six underlined diseases are investigated by the Specific Disease Study Group of the MHLW. Polyarteritis nodosa and microscopic polyangiitis were categorized collectively as "periarteritis nodosa" until 2005. From 2006 on, these diseases have been clearly distinguished. ANCA, antineutrophil cytoplasmic antibodies; MHLW, Ministry of Health, Labour and Welfare. Wegener's granulomatosis Buerger disease Takayasu arteritis Malignant rheumatoid arthritis Microscopic polyangiitis + Polyarteritis nodosa