treatment
Last edited 05/2020 and last reviewed 09/2022
Seek expert advice.
Management
- induction treatment for most patients with ANCA associated vasculitis (AAV) should be with cyclophosphamide or rituximab and glucocorticoids
- AAV should be considered to be a chronic disease needing longterm immunosuppressive therapy
- rituximab should be considered as an alternative induction agent for those at high risk of infertility and infection
- mortality remains high, and late death is due to cardiovascular disease, infection (secondary to treatment) and malignancy
Notes:
- role of serial ANCA measurement in determining treatment during remission remains controversial
- some current evidence suggests patients in whom ANCA remains present or rises more than fourfold are at greater risk of relapse.
Reference:
- Davies DJ. Small vessel vasculitis. Cardiovascular Pathology 2005; 14 (6): 335-346.
- ARC Autumn 2012. Topical Reviews - ANCA-associated vasculitis; 1:1-12.
- Yates A, Watts R. ANCA-associated vasculitis. Clinical Medicine 2017 Vol 17, No 1: 60–4
avacopan for treating severe active granulomatosis with polyangiitis or microscopic polyangiitis