Abstract

This special edition reviews the progress in understanding of systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens (ANCA). European research groups have contributed to this research including the original observations of the association between ANCA and vasculitis. Areas of interest include classification and epidemiology, genetics and pathogenesis, disease assessment, histology and long-term outcomes. Clinical trials conducted by the European Vasculitis Study group have helped to define the current standard of care for the treatment of patients with vasculitis and provided a platform for the investigation of newer therapies. The prognosis of patients with ANCA-associated vasculitis has improved over this period as a result of facilitated diagnosis and development of consensus, evidence-based, treatment recommendations. The ANCA story represents an example of the power of a biomarker in influencing a disease area, inspiring research and providing physicians with better tools to treat patients with these disorders.

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