Eosinophilic granulomatosis with polyangiitis: recent therapeutic advances

The aim of this review is to describe the substantial advances that have been made in the past 2 years on new treatment options in eosinophilic granulomatosis with polyangiitis (EGPA).

The therapeutic scenario in EGPA has been recently broadened by the publication of the results from the multicenter, double-blind randomized MANDARA trial, which proved the noninferiority of benralizumab to another anti-interleukin-5 (IL-5) drug, mepolizumab, for the induction of remission in patients with EGPA. A few real-world studies have confirmed these results. Furthermore, the first randomized controlled trial exploring the efficacy of rituximab (anti-CD20) in induction of remission of EGPA was recently published. Targeting other molecular pathways did not always show adequate control of systemic manifestations of EGPA, with only limited evidence of effectiveness from small case series. Interestingly, cases of EGPA onset in severe asthma patients treated with monoclonal antibodies were described.

Biological drug therapy targeting IL-5 consolidated its role in the management of EGPA, becoming the cornerstone of the treatment of this rare disease. Future guidelines should consider these recent findings to improve the management of EPGA. Notably, while selective IL-5 targeting is highly effective for remission maintenance, its role in inducing remission in EGPA remains to be fully established. Rituximab was non-superior to standard therapy in induction of remission in patients with EGPA, demonstrating a similar rate of response. The role of other targeted therapies, albeit promising in some cases, remains a matter of debate.