Objective In 2020, the Canadian Vasculitis Research Network (CanVasc) published their updated recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). The current addendum provides further recommendations regarding the use of avacopan in AAV based on a review of newly available evidence. Methods An updated systematic literature review on avacopan (formerly, CCX168) using Medline, Embase, and the Cochrane Library was performed for publications up to September 2022. New recommendations were developed and categorized according to the EULAR grading levels, as done for previous CanVasc recommendations. A modified Delphi procedure and videoconferences were used to reach ≥80% consensus on the inclusion, wording and grading of each recommendation. Results Three new recommendations were developed. They focus on avacopan therapy indication and duration, as well as timely glucocorticoid tapering. Conclusion These 2022 addended recommendations provide rheumatologists, nephrologists, and other specialists caring for patients with AAV with guidance for the use of avacopan, based on current evidence and consensus from Canadian experts.

Purpose of review Interstitial lung disease (ILD) is now recognized as a common complication of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), especially myeloperoxidase (MPO)-ANCA-positive AAV and microscopic polyangiitis (MPA). This review focuses on current concepts pertaining to the pathogenesis, clinical assessment, and management of AAV-ILD. Recent findings ILD is typically identified before or at the onset of systemic AAV, and usual interstitial pneumonia (UIP) is the most common CT pattern. MPO-ANCA production, neutrophil extracellular traps formation, reactive oxidative species production, complement activation, environmental exposures, and genetic background might play a role in the pathogenesis of AAV-ILD. Recent research has identified promising biomarkers as potential diagnostic and prognostic tools in AAV-ILD. The optimal treatment for AAV-ILD is not well defined but might rely on a combination of immunosuppression and antifibrotics, especially in patients with progressive lung fibrosis. Despite the effectiveness of current therapies for AAV, the outcome of patients with AAV-ILD remains poor. Summary ANCA screening should be considered in patients with newly diagnosed ILD. Management of AAV-ILD should be overviewed by a collaborative team comprising vasculitis experts and respirologists. Graphical abstract http://links.lww.com/COPM/A33