Current Diagnosis and Treatment of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Review Including a Comparison of Characteristics in Europe and Japan.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a disease entity characterized by systemic vasculitis positive for ANCAs, which often leads to severe organ damage such as diffuse bronchoalveolar hemorrhage and rapidly progressive glomerulonephritis. It is known that the incidence and characteristics of AAV vary depending on region, and differences in the peak age of onset, the ratio of positive rates of MPO-ANCA to PR3-ANCA, and occurrence rates of GPA and MPA may have resulted in different approaches to clinical practice. It may also be necessary to modify therapeutic strategies according to ethnic factors. Avacopan is a therapeutic option recently recommended for the management of AAV; however, the rate of severe liver injuries associated with avacopan was reported to be relatively high in the Japanese population. In this review, we introduce current globally recognized knowledge on the diagnosis and treatment of AAV, including a comparison of patient characteristics and clinical practice between Europe and Japan obtained from the recent literature.