management

Last edited 10/2020 and last reviewed 09/2022

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
• molecules other than anti- TNF agents and rituximab, such as abatacept, mepolizumab (an anti-IL5 antibody) and alemtuzumab (a humanised monoclonal
  anti-CD52 antibody) have been used in refractory cases of AAV (4)
• a systematic review "...found moderate-certainty evidence that in patients with relapsing or refractory EGPA, mepolizumab compared to placebo probably
  decreases disease relapse and low-certainty evidence that mepolizumab may increase the probability of accruing at least 24 weeks of
  disease remission..."

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
• Bala MM et al. Anti-cytokine targeted therapies for ANCA-associated vasculitis. Cochrane Database of Systematic Reviews 2020, Issue 9. Art. No.: CD008333.
  DOI: 10.1002/14651858.CD008333.pub2.