Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis

In the case of a clinical presentation compatible with small-vessel vasculitis in combination with positive myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA serology, waiting for a kidney biopsy to be performed or reported should not delay starting immunosuppressive therapy, especially in patients who are rapidly deteriorating (Figure 1). (, )

We recommend that glucocorticoids in combination with rituximab or cyclophosphamide be used as initial treatment of new-onset AAV. (1, B)