This article presents the updated 2015 recommendations for the management of ANCA-associated vasculitis (AAV) from the European League Against Rheumatism (EULAR), the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based on systematic literature reviews and expert opinion, with a focus on remission induction and maintenance, treatment options, and long-term complications. Key recommendations include: 1. **Remission Induction**: For organ-threatening or life-threatening AAV, a combination of glucocorticoids and either cyclophosphamide or rituximab is recommended. For non-organ-threatening AAV, a combination of glucocorticoids and methotrexate or mycophenolate mofetil is recommended. 2. **Plasma Exchange**: Plasma exchange is recommended for patients with rapidly progressive glomerulonephritis or severe diffuse pulmonary hemorrhage. 3. **Remission Maintenance**: Treatment with low-dose glucocorticoids and azathioprine, rituximab, methotrexate, or mycophenolate mofetil is recommended. 4. **Refractory Disease**: For patients unresponsive to standard therapy, switching between cyclophosphamide and rituximab is recommended. 5. **Clinical Assessment**: Structured clinical assessment, rather than ANCA testing, should guide treatment changes. 6. **Long-term Follow-up**: Patients should be assessed for comorbidities and provided with necessary therapies or support. The recommendations aim to provide a comprehensive framework for the management of AAV, emphasizing the importance of shared decision-making and long-term care.This article presents the updated 2015 recommendations for the management of ANCA-associated vasculitis (AAV) from the European League Against Rheumatism (EULAR), the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based on systematic literature reviews and expert opinion, with a focus on remission induction and maintenance, treatment options, and long-term complications. Key recommendations include: 1. **Remission Induction**: For organ-threatening or life-threatening AAV, a combination of glucocorticoids and either cyclophosphamide or rituximab is recommended. For non-organ-threatening AAV, a combination of glucocorticoids and methotrexate or mycophenolate mofetil is recommended. 2. **Plasma Exchange**: Plasma exchange is recommended for patients with rapidly progressive glomerulonephritis or severe diffuse pulmonary hemorrhage. 3. **Remission Maintenance**: Treatment with low-dose glucocorticoids and azathioprine, rituximab, methotrexate, or mycophenolate mofetil is recommended. 4. **Refractory Disease**: For patients unresponsive to standard therapy, switching between cyclophosphamide and rituximab is recommended. 5. **Clinical Assessment**: Structured clinical assessment, rather than ANCA testing, should guide treatment changes. 6. **Long-term Follow-up**: Patients should be assessed for comorbidities and provided with necessary therapies or support. The recommendations aim to provide a comprehensive framework for the management of AAV, emphasizing the importance of shared decision-making and long-term care.