What is your second line therapy for patients with EGPA with mainly pulmonary and sinonasal features who did not respond to mepolizumab 300 mg/month and still require high doses of steroid?

This is an important question.

Benralizumab has recently been shown to have similar efficacy to mepolizumab with a suggestion of a greater number of patients being able to fully discontinue steroids when treated with benralizumab as compared to mepolizumab. On the basis of this study I would use benralizumab in this patient population who have failed mepolizumab.

If that is ineffective, the choice of the next agent is uncertain. Briefly summarizing the available evidence: Methotrexate and azathioprine have been utilized frequently with anecdotal and case series benefit, however, there is no randomized trial data for their efficacy. Rituximab has been investigated in the REOVAS study, currently only reported in abstract format, where it was s...

Guidelines for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are based on limited data from clinical trials or even protocol-based cohorts. However, there are several ongoing and planned randomized controlled trials that will likely help inform new approaches to treating this complex disease. Although I advise avoiding overly seeking to categorize manifestations of EGPA according to the problematic dichotomy of the “vasculitis” vs. “eosinophilic” labels, it is imperative to recognize the need to address reactive airway disease (asthma) based on additional information available from the vastly larger database for non-EGPA asthma. Thus, the first approach to a patient with difficult-to-treat asthma, which is quite comm...