Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune-mediated inflammation of small and medium-sized vessels that can affect virtually any organ system and bears the risk of irreversible organ damage. Without treatment the mortality rates are high, which necessitates rapid diagnosis and initiation of treatment. Histological confirmation, which is not feasible in all cases, should be strived for, especially to delineate differential diagnoses and vasculitis mimics. The new American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria are primarily designed for study purposes and show limitations in the routine application. Globally, the recently updated EULAR recommendations represent the most up to date management guidelines. Therapeutically, rituximab and cyclophosphamide in combination with glucocorticoids remain the pillars of treatment in remission induction for severe organ-threatening and life-threatening diseases. For the first time, mepolizumab and avacopan represent approved treatment options for specific entities that make a significant contribution to steroid reduction. New attention has been paid to patient-reported outcomes, for which adisease-specific outcome questionnaire is now available.
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