ANCA-Associated Vasculitis Induction, Maintenance, and Relapse Treatment

Authored by Sourabh Sharma, published on 2026-11-12 14:04:26.0

  1. Confirm AAV; Define PR3/MPO Status and Kidney, Pulmonary, Neurologic, Systemic Involvement
    • Screen HBV/IgG, Update Vaccines, Preserve Fertility, Monitor Toxicity; Plan PJP Prophylaxis for Rituximab, Cyclophosphamide, or Prolonged High-Dose GC
      • GPA/MPA or EGPA?
        • Life- or Organ-Threatening GPA/MPA?
          • Start Rituximab or Methotrexate Induction
            • Use Reduced-Dose GC Taper or Avacopan-Based GC-Sparing Strategy
              • Selective Plasma Exchange Indication Present?
                • Add Plasma Exchange to Induction
                  • Clinical Remission Achieved After Induction?
                    • Assess Relapse Risk, Prior Therapy, Infection Risk, and Toxicity
                      • Start Rituximab; Use Azathioprine When Rituximab Is Unsuitable
                        • Individualize Maintenance Duration From 18 Months–4 Years
                          • Relapse or Persistent Active Disease?
                            • Treat Relapse by Severity; Prefer Rituximab for Major Relapse
                            • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                            • Stop Maintenance When Appropriate; Continue Clinical and Kidney Surveillance
                    • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                • Clinical Remission Achieved After Induction?
                  • Assess Relapse Risk, Prior Therapy, Infection Risk, and Toxicity
                    • Start Rituximab; Use Azathioprine When Rituximab Is Unsuitable
                      • Individualize Maintenance Duration From 18 Months–4 Years
                        • Relapse or Persistent Active Disease?
                          • Treat Relapse by Severity; Prefer Rituximab for Major Relapse
                          • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                          • Stop Maintenance When Appropriate; Continue Clinical and Kidney Surveillance
                  • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
          • Dialysis, SCr >4 mg/dL, or Rapidly Declining Kidney Function?
            • Start Rituximab or Cyclophosphamide Induction
              • Use Reduced-Dose GC Taper or Avacopan-Based GC-Sparing Strategy
                • Selective Plasma Exchange Indication Present?
                  • Add Plasma Exchange to Induction
                    • Clinical Remission Achieved After Induction?
                      • Assess Relapse Risk, Prior Therapy, Infection Risk, and Toxicity
                        • Start Rituximab; Use Azathioprine When Rituximab Is Unsuitable
                          • Individualize Maintenance Duration From 18 Months–4 Years
                            • Relapse or Persistent Active Disease?
                              • Treat Relapse by Severity; Prefer Rituximab for Major Relapse
                              • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                              • Stop Maintenance When Appropriate; Continue Clinical and Kidney Surveillance
                      • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                  • Clinical Remission Achieved After Induction?
                    • Assess Relapse Risk, Prior Therapy, Infection Risk, and Toxicity
                      • Start Rituximab; Use Azathioprine When Rituximab Is Unsuitable
                        • Individualize Maintenance Duration From 18 Months–4 Years
                          • Relapse or Persistent Active Disease?
                            • Treat Relapse by Severity; Prefer Rituximab for Major Relapse
                            • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                            • Stop Maintenance When Appropriate; Continue Clinical and Kidney Surveillance
                    • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
            • Start Cyclophosphamide; Use Rituximab–Cyclophosphamide Combination in Selected Patients
              • Use Reduced-Dose GC Taper or Avacopan-Based GC-Sparing Strategy
                • Selective Plasma Exchange Indication Present?
                  • Add Plasma Exchange to Induction
                    • Clinical Remission Achieved After Induction?
                      • Assess Relapse Risk, Prior Therapy, Infection Risk, and Toxicity
                        • Start Rituximab; Use Azathioprine When Rituximab Is Unsuitable
                          • Individualize Maintenance Duration From 18 Months–4 Years
                            • Relapse or Persistent Active Disease?
                              • Treat Relapse by Severity; Prefer Rituximab for Major Relapse
                              • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                              • Stop Maintenance When Appropriate; Continue Clinical and Kidney Surveillance
                      • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                  • Clinical Remission Achieved After Induction?
                    • Assess Relapse Risk, Prior Therapy, Infection Risk, and Toxicity
                      • Start Rituximab; Use Azathioprine When Rituximab Is Unsuitable
                        • Individualize Maintenance Duration From 18 Months–4 Years
                          • Relapse or Persistent Active Disease?
                            • Treat Relapse by Severity; Prefer Rituximab for Major Relapse
                            • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
                            • Stop Maintenance When Appropriate; Continue Clinical and Kidney Surveillance
                    • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
        • Life- or Organ-Threatening EGPA?
          • Start GC Plus Cyclophosphamide; Use Rituximab Selectively
            • EGPA Remission Achieved After Induction?
              • Individualize EGPA Maintenance; Treat Relapse by Phenotype and Severity
              • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
          • Which Manifestations Drive Non-Organ-Threatening EGPA Treatment?
            • Start GC Plus Mepolizumab for Eosinophilic or Airway-Predominant EGPA
              • EGPA Remission Achieved After Induction?
                • Individualize EGPA Maintenance; Treat Relapse by Phenotype and Severity
                • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
            • Start GC With Steroid-Sparing Immunosuppression
              • EGPA Remission Achieved After Induction?
                • Individualize EGPA Maintenance; Treat Relapse by Phenotype and Severity
                • Reassess Diagnosis, Activity, Adherence, and Exposure; Switch Induction Agent
tosprivacy