This review discusses current thoughts on the pathogenesis of vasculitis. Secondary vasculitides, frequently associated with infections or systemic autoimmune diseases, are, in most cases, characterized by immune deposits in the vessel wall, which probably underlies the development of lesions. In the primary vasculitides, immune deposits are generally absent. A group of primary vasculitides is, however, strongly associated with anti-neutrophil cytoplasmic autoantibodies (ANCA). Various in vitro and in vivo experimental data suggest that those ANCA are involved in the pathogenesis of lesions in the associated disorders.