The EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis (AAV) provide updated guidelines for the treatment of this group of vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). These recommendations are based on systematic literature reviews and expert opinion, with levels of evidence and grades of recommendation determined through consensus and voting. The guidelines emphasize the importance of shared decision-making between patients and clinicians, and cover key aspects of treatment, including remission induction, maintenance, and relapse management. For remission induction in organ-threatening or life-threatening AAV, a combination of glucocorticoids and either cyclophosphamide or rituximab is recommended. Cyclophosphamide and rituximab are considered equally effective for this purpose. For non-organ-threatening AAV, glucocorticoids combined with methotrexate or mycophenolate mofetil are recommended. For remission maintenance, low-dose glucocorticoids combined with azathioprine, rituximab, methotrexate, or mycophenolate mofetil are recommended. The recommendations also emphasize the importance of long-term follow-up and the need for patients to be managed in centres of expertise. Plasma exchange is recommended for patients with rapidly progressive glomerulonephritis or severe diffuse pulmonary haemorrhage. The use of rituximab is preferred over cyclophosphamide for relapsing disease, particularly in patients who wish to preserve reproductive potential. Cyclophosphamide is associated with reduced ovarian reserve and male infertility, while the long-term effects of rituximab on fertility are not well studied. The recommendations also address the importance of structured clinical assessments rather than ANCA testing for treatment decisions, the need for periodic assessment of cardiovascular risk, and the investigation of persistent unexplained haematuria in patients with prior exposure to cyclophosphamide. Hypoimmunoglobulinaemia after rituximab treatment is recommended to be tested, and patients with AAV should be immunised against infectious diseases according to local policy. The guidelines also emphasize the importance of managing comorbidities associated with AAV and the need for patients to be informed about their disease, treatment options, and long-term prognosis. The recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries, and drug regulatory organisations. The guidelines are based on a multidisciplinary approach and include inputs from rheumatologists, internists, renal physicians, and other specialists. The recommendations are subject to periodic review and updating due to ongoing research and advances in the field.The EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis (AAV) provide updated guidelines for the treatment of this group of vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). These recommendations are based on systematic literature reviews and expert opinion, with levels of evidence and grades of recommendation determined through consensus and voting. The guidelines emphasize the importance of shared decision-making between patients and clinicians, and cover key aspects of treatment, including remission induction, maintenance, and relapse management. For remission induction in organ-threatening or life-threatening AAV, a combination of glucocorticoids and either cyclophosphamide or rituximab is recommended. Cyclophosphamide and rituximab are considered equally effective for this purpose. For non-organ-threatening AAV, glucocorticoids combined with methotrexate or mycophenolate mofetil are recommended. For remission maintenance, low-dose glucocorticoids combined with azathioprine, rituximab, methotrexate, or mycophenolate mofetil are recommended. The recommendations also emphasize the importance of long-term follow-up and the need for patients to be managed in centres of expertise. Plasma exchange is recommended for patients with rapidly progressive glomerulonephritis or severe diffuse pulmonary haemorrhage. The use of rituximab is preferred over cyclophosphamide for relapsing disease, particularly in patients who wish to preserve reproductive potential. Cyclophosphamide is associated with reduced ovarian reserve and male infertility, while the long-term effects of rituximab on fertility are not well studied. The recommendations also address the importance of structured clinical assessments rather than ANCA testing for treatment decisions, the need for periodic assessment of cardiovascular risk, and the investigation of persistent unexplained haematuria in patients with prior exposure to cyclophosphamide. Hypoimmunoglobulinaemia after rituximab treatment is recommended to be tested, and patients with AAV should be immunised against infectious diseases according to local policy. The guidelines also emphasize the importance of managing comorbidities associated with AAV and the need for patients to be informed about their disease, treatment options, and long-term prognosis. The recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries, and drug regulatory organisations. The guidelines are based on a multidisciplinary approach and include inputs from rheumatologists, internists, renal physicians, and other specialists. The recommendations are subject to periodic review and updating due to ongoing research and advances in the field.