Updated guidelines on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were released in 2021 by the American College of Rheumatology jointly with the Vasculitis Foundation and, subsequently, in 2022 by the European Alliance of Associations for Rheumatology. In addition, in 2021, the Kidney Disease: Improving Global Outcomes had released updated recommendations on the treatment of AAV with glomerulonephritis (AAV-GN). Kidney involvement is particularly relevant in microscopic polyangiitis and granulomatosis with polyangiitis, but is less frequent in eosinophilic granulomatosis with polyangiitis. The management of AAV-GN has been a focus for drug development and change over the past 10 years. Avoidance of progression to end-stage kidney disease (ESKD) or kidney failure is one of the main unmet needs in the management of AAV, with ESKD having a major impact on morbidity, health costs and mortality risk. Relevant changes in AAV-GN management are related to remission-induction treatment of patients with severe kidney disease, the use of glucocorticoids and avacopan, and remission-maintenance treatment. All the documents provide guidance in accordance with the evidence-based standard of care available at the time of their release. With our work we aim to (i) show the progress made and identify the differences between guidelines and recommendations, (ii) discuss the supporting rationale for those, and (iii) identify gaps in knowledge that could benefit from additional research and should be revised in subsequent updates.

• Keywords
• ANCA-associated vasculitis, glomerulonephritis, granulomatosis with polyangiitis, guidelines, microscopic polyangiitis,
• MeSH
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis * complications   drug therapy   MeSH
• Kidney Failure, Chronic * etiology   therapy   MeSH
• Churg-Strauss Syndrome * MeSH
• Glomerulonephritis * drug therapy   MeSH
• Granulomatosis with Polyangiitis * therapy   MeSH
• Humans MeSH
• Microscopic Polyangiitis * therapy   MeSH
• Antibodies, Antineutrophil Cytoplasmic MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Names of Substances
• Antibodies, Antineutrophil Cytoplasmic MeSH