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