Management

Management (1)

• 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 long term 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 (2)
  • 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..."

References:

1. Chakraborty R et al. Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome).Treasure Island (FL): StatPearls Publishing. 2024.
2. Bala MM et al. Anti-cytokine targeted therapies for ANCA-associated vasculitis. Cochrane Database of Systematic Reviews 2020, Issue 9. Art. No.: CD008333.