Abstract
Diagnosing and managing antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remain a challenge for many clinicians, due to the complexity of the disease manifestations and its treatment. There has been a paradigm shift in ANCA vasculitis management, where treatment incorporates both emergency life- and organ-saving procedures and longer-term care to manage relapse and co-morbidity risk and the complications of organ damage. Here, we highlight 10 key tips for the management of ANCA-associated vasculitis based on current evidence and clinical experience. First, we advise making the diagnosis as early as possible, emphasizing the importance of using high-quality ANCA assays. Second, we recommend the use of glucocorticoids in combination with rituximab and/or cyclophosphamide as induction therapy. Third, plasma exchange should be considered in patients with severe renal impairment and diffuse alveolar haemorrhage. We advise the use of rapidly reducing glucocorticoid regimens and advocate consideration of avacopan early in the disease course. We recommend the use of rituximab as maintenance therapy and routine monitoring of kidney function, proteinuria, ANCA and immunoglobulin levels at baseline and during follow-up. The use of prophylactic antibiotics in susceptible patients and timely vaccination schedules is discussed. Rituximab is the preferred immune suppressive for treatment of relapse. Finally, we recommend switching treatment modalities in patients whose vasculitis is refractory to induction therapy and to consider plasma exchange in selected patients. These key tips aim to provide the necessary guidance to improve patient outcomes and reduce adverse events.