What Has Changed in the New Guidelines in the Treatment of ANCA-Associated Vasculitis?

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rapidly progressing disease with high mortality and morbidity. There is necrotizing inflammatory involvement in small vessels associated with autoantibodies against proteinase-3 (PR3) and myeloperoxidase (MPO-ANCA). Mortality rates are significantly higher within the first year. Causes that increase mortality include rapid deterioration in serum creatinine and glomerular filtration rate, pulmonary hemorrhage, delay in treatment, and adverse events related to treatments. Kidney and other organ involvement also affect the treatment protocol and dose. Kidney Diseases Improving Global Outcomes (KDIGO) has been providing treatment management guidelines for glomerulonephritis, including AAV-related glomerular involvement, since 2012. The most recently published glomerulonephritis treatment guideline in 2021 highlights the emphasis on rapid reduction of the glucocorticoid dose through controlled studies. With the approval of the complement 5a antagonist avacopan (CCX 168) in AAV, KDIGO has recently published separate guidance for AAV treatment management. This article discusses AAV treatment recommendations and changes in the 2024 guideline.