A multicenter retrospective study of 92 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) who received avacopan showed high remission rates and an acceptable safety profile. Avacopan, an oral complement component 5a receptor blocker, was used in combination with rituximab and low-dose cyclophosphamide in 47% of patients and with plasma exchange in 14%. Clinical remission was achieved in 90% of patients at week 26 and 84% at week 52. The mean estimated glomerular filtration rate (eGFR) increased significantly over time, with a mean increase of +12.2 ml/min per 1.73 m² at week 26 and +19.8 ml/min per 1.73 m² at week 52. Patients with lower baseline eGFR showed greater improvements. Avacopan was generally well-tolerated, with 20% of patients discontinuing due to adverse events, primarily elevated serum aminotransferases. The study included patients with advanced kidney disease and those on dialysis, populations excluded from the ADVOCATE trial. Avacopan was initiated within 30 days of induction in 60% of patients, with those starting earlier showing better outcomes. However, delays in avacopan initiation were common, with a median of 3 weeks from induction. The study highlights the effectiveness of avacopan in real-world settings, including populations not studied in clinical trials. The safety profile was acceptable, with the most common adverse event being elevated aminotransferases. The study also noted variations in steroid use and treatment strategies, emphasizing the need for further research to confirm the efficacy and safety of avacopan in combination therapies. Overall, avacopan demonstrated high remission rates and acceptable safety, supporting its use in AAV treatment.A multicenter retrospective study of 92 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) who received avacopan showed high remission rates and an acceptable safety profile. Avacopan, an oral complement component 5a receptor blocker, was used in combination with rituximab and low-dose cyclophosphamide in 47% of patients and with plasma exchange in 14%. Clinical remission was achieved in 90% of patients at week 26 and 84% at week 52. The mean estimated glomerular filtration rate (eGFR) increased significantly over time, with a mean increase of +12.2 ml/min per 1.73 m² at week 26 and +19.8 ml/min per 1.73 m² at week 52. Patients with lower baseline eGFR showed greater improvements. Avacopan was generally well-tolerated, with 20% of patients discontinuing due to adverse events, primarily elevated serum aminotransferases. The study included patients with advanced kidney disease and those on dialysis, populations excluded from the ADVOCATE trial. Avacopan was initiated within 30 days of induction in 60% of patients, with those starting earlier showing better outcomes. However, delays in avacopan initiation were common, with a median of 3 weeks from induction. The study highlights the effectiveness of avacopan in real-world settings, including populations not studied in clinical trials. The safety profile was acceptable, with the most common adverse event being elevated aminotransferases. The study also noted variations in steroid use and treatment strategies, emphasizing the need for further research to confirm the efficacy and safety of avacopan in combination therapies. Overall, avacopan demonstrated high remission rates and acceptable safety, supporting its use in AAV treatment.