Anti-neutrophil cytoplasmic antibody-associated vasculitis, large vessel vasculitis and Kawasaki disease in Japan.

Based on studies comparing the prevalence of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) between Japan and Europe, we have learned that the difference may be due to genetic background and environmental factors, but not to diagnosis or ELISA system for myeloperoxidase and proteinase-3 ANCA. In Japan, microscopic polyangiitis is the most common among AAV, but Wegener's granulomatosis was present in less than 2 per million patients. Also, one study from Hokkaido reported only 16 patients in a 27-year time frame. A recent retrospective study of renal vasculitis between 2000 and 2004 from Miyazaki prefecture in Japan reported an incidence of microscopic polyangiitis of 14.8 per million, but no patients with Wegener's granulomatosis or Churg-Strauss syndrome. In the present review, we focus on ANCA-related vasculitis in Japan: (1) AAV and large vessel vasculitis - Takayasu's arteritis and giant cell arteritis; (2) primary renal vasculitis; (3) epitopes of myeloperoxidase-ANCA in vasculitis in the Japanese population and comparison of ANCA-ELISA systems in Japan and Europe, and finally (4) children with vasculitis in Japan involving Kawasaki disease - a systemic vasculitis.