Recent advances in the management of antineutrophil cytoplasmic antibody-associated vasculitis

The prognosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has remarkably improved from an almost uniformly fatal disease about three decades back, to a chronic manageable disease with survival rates in excess of 80%. Induction of remission largely relies on cyclophosphamide or rituximab-based regimens, although emerging data suggest some potential role of mycophenolate mofetil (MMF), especially in milder disease phenotypes. Recent emphasis has shifted to exploration of treatment regimens minimizing dosage and duration of corticosteroid exposure, or replacing corticosteroids altogether with agents like avacopan, in an effort to ameliorate risks due to glucocorticoids as well as minimize damage accrual. Maintenance regimens traditionally have used azathioprine, methotrexate, or less commonly, MMF. However, rituximab is emerging as a useful maintenance agent, with the advantage of single-dose administration 6 monthly. Dosing interval for rituximab maintenance is another area of active research, with emerging literature suggesting the effectiveness of a dosing schedule based on B-cell repopulation or rise in ANCA titers. Long-term considerations for patients with AAV include the risk of infections (which can be minimized with alternate-day cotrimoxazole therapy), hypogammaglobulinemia in patients on rituximab, and accrual of damage, including drug-related damage (such as diabetes and osteoporosis) and malignancies. Ongoing clinical trials attempt to decipher the role of plasma exchange, as well as delineate the exact role of newer steroid-sparing therapeutic modalities such as avacopan. Recent clinical guidelines for the management of AAV from multiple societies, including the European League against Rheumatism and the British Society for Rheumatology, provide common treatment recommendations for AAV management.