Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a heterogeneous group of rare diseases characterised by necrotising inflammation of the small blood vessels and the presence of ANCA with specificity for proteinase-3 or myeloperoxidase. Genetic susceptibility along with malignancy, drug exposure, and environmental exposures to infectious agents and silica are involved in disease progression. To date, growing evidence has revealed that ANCA specificity defines homogeneous groups of patients more effectively than clinical diagnosis, since proteinase-3 ANCA and myeloperoxidase-ANCA are linked with different genetic backgrounds and epidemiologies. This review presents current and updated knowledge on the central aetiopathogenic role of genetic associations and environmental exposures in AAV; discusses the main mechanisms of ANCA immunogenesis; and highlights the value of ANCA specificity for future classification criteria.
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