Substantial progress has been made over the last two
decades in our understanding of the immunopathogenesis of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides. Compelling evidence from in vitro studies and experimental models in conjunction with clinical trials has confirmed that ANCA directly contribute to the evolution and progression of the disease process. Continuous development in our understanding of the mechanisms
that drive the disease may ultimately allow us to tailor the multitude of novel therapies, which are rapidly becoming available, to the requirements of individual patients. In this review we endeavour to provide a brief overview of the recent advances in ANCA-associated vasculitides and outline basic principles for diagnosis and treatment of these complex multisystem diseases.