Updated treatment approaches for eosinophilic granulomatosis with polyangiitis: A systematic scoping review.

Abstract

Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis causing multi-organ damage and long-term disability. Various treatments including IL-5-targeting agents and other biologics have recently emerged, prompting regular updates of clinical practice guidelines. This scoping review and systematic review (SR) aimed to evaluate the efficacy and safety of rituximab and benralizumab in EGPA.

Methods: A scoping review was conducted to identify relevant clinical questions, followed by an SR of trials published between December 2018 and July 2023. Studies comparing rituximab or benralizumab with standard treatments were included. Risk of bias (RoB) was assessed using RoB 2 and quality of evidence was rated using GRADE.

Results: One randomised controlled trial (RCT; n = 105) comparing rituximab with conventional therapy, showed a 180-day remission rate of 63.5% versus 60.4% (risk ratio: 1.05, 95% CI: 0.78-1.42), with very low certainty due to serious RoB and imprecision. Another RCT compared benralizumab with mepolizumab, showing similar rates of remission (58.6% versus 55.7%), relapse (30%), and glucocorticoid tapering, with low certainty owing to imprecision.

Conclusions: Rituximab showed no clear benefit over conventional therapy, whereas benralizumab demonstrated comparable efficacy and safety to mepolizumab. However, evidence remains limited, and further EGPA-specific trials are needed.