Many classifications of vasculitis syndrome have been proposed. The American College of Rheumatology (ACR) proposed criteria for classification of vasculitis in 1990. In 1994, the nomenclature of systemic vasculitides was proposed by Chapel Hill International Consensus Conference. In this conference, the nomenclature of systemic vasculitides was discussed and confirmed by the committee consisted of 16 investigators from 6 countries. The classification of systemic vasculitides was proposed in concern with the size of involved artery and also with the appearance of antineutrophil cytoplasmic antibodies (ANCA). In Japan, Research committee of intractable vasculitis syndrome, Survey and Research on Specific Diseases, the Ministry of Health and Welfare of Japan proposed diagnostic criteria for vasculitis syndrome. The criteria for ANCA related vasculitis syndrome, temporal arteritis and antiphospholipid syndrome were proposed and a nation-wide survey has been undertaken in this committee (Chairman: Hiroshi Hashimoto). In this review, we describe a recent classification of vasculitides and recent topics on Takayasu arteritis, temporal arteritis, Wegener's granulomatosis and Churg-Strauss syndrome.