ANCA-associated vasculitis (AAV) is a systemic, potentially organ and life threatening chronic autoimmune disease. With current management strategies, such as high-dose glucocorticoids in combination with cyclophosphamide or rituximab, outcomes have progressively improved with overall remission rates of 70-90%. However, relapse rates after discontinuation of therapy are consistently high, and treatment-related toxicity, mainly driven by glucocorticoids, still determines morbidity and quality of life. Prevention of relapses while minimizing adverse events is a major goal of long-term treatment, but the optimal duration of maintenance therapy and the role and utility of glucocorticoids in this context remains controversial. This review of induction and maintenance treatment of AAV aims to offer practical advice on immunosuppressive therapies and patient care, addressing individual risk factors and their therapeutic implications. It will discuss benefits and harms of the use of glucocorticoids, particularly focusing on recent advances in steroid sparing concepts.
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