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.

Department of Health Medicine and Caring Sciences Linköping University Linköping Sweden

Department of Internal Medicine 4 Hospital of the Ludwig Maximilians University Munich Germany

Department of Internal Medicine 4 Medical University Innsbruck Innsbruck Austria

Department of Medicine University of Cambridge Cambridge United Kingdom

Department of Nephrology 1st Faculty of Medicine and General University Hospital Charles University Prague Czechia

Department of Nephrology and Renal Transplantation Patras University Hospital Patras Greece

Department of Nephrology Hospital Universitario La Paz IdiPAZ Madrid Spain

Department of Nephrology Limassol General Hospital SHSO Cyprus; Medical School University of Nicosia Nicosia Cyprus

Department of Renal Medicine Karolinska University Hospital and CLINTEC Karolinska Institutet Stockholm Sweden

Division of Nephrology and Hypertension Department of Medicine Mayo Clinic College of Medicine and Science Rochester MN USA

Division of Nephrology and Transplant Medicine Department of Medicine Leiden University Medical Center Leiden The Netherlands

Division of Nephrology and Transplantation Queen Elizabeth University Hospital Glasgow United Kingdom

Division of Nephrology Department of Clinical Sciences Lund Lund University and Skane University Hospital Lund Sweden

Division of Nephrology Department of Internal Medicine Necmettin Erbakan University Konya Turkey

Division of Nephrology Department of Medicine Johns Hopkins University School of Medicine Baltimore MD United States of America

Division of Nephrology RWTH Aachen University Hospital Aachen Germany

Division of Pulmonary and Critical Care Medicine Department of Medicine Mayo Clinic College of Medicine and Science Rochester MN USA

Faculdade de Medicina da Universidade do Porto Departamento de Biomedicina Porto Portugal

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