Advances in the pharmacotherapeutic management of eosinophilic granulomatosis with polyangiitis.

INTRODUCTION

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare but potentially lethal systemic vasculitis. Only a few prospective therapeutic trials had been conducted in EGPA, and its treatment was mostly adapted from other vasculitides. Monoclonal antibodies inhibiting various pathways (e.g. interleukin-5 [IL5] or B cells) have been investigated.

AREAS COVERED: Published studies on treatments for EGPA using glucocorticoids, conventional immunosuppressants (such as cyclophosphamide or azathioprine), antiIL5 pathway agents (mepolizumab, approved by the Food and Drug Administration (FDA) and European Medicines Agency (EMA) for EGPA; benralizumab and reslizumab), other and future possible treatments [PubMed search, 01/1990-02/2023] are reviewed.

EXPERT OPINION: With advances made in the pharmacotherapeutic management of EGPA, the prognosis has gradually shifted from a potentially fatal to a more chronic course, for which more targeted and safer treatments can be used. However, glucocorticoids remain central. Rituximab is now a possible alternative to cyclophosphamide for induction, although data are still limited. AntiIL5 pathway therapies have been shown to be safe and effective in relapsing patients with EGPA, who often experience asthma and/or ears, nose, and throat (ENT) manifestations, but long-term data are needed. Treatment strategies need to be optimized based on individual patient characteristics, likely with sequential, combination-based approaches, while topical airway treatments should not be forgotten.