EGPA Without Organ-Threatening Manifestations: What to Do When Glucocorticoids Do Not Achieve Remission

This protocol applies to patients with eosinophilic granulomatosis with polyangiitis (EGPA) presenting without organ-threatening or life-threatening manifestations, in whom initial glucocorticoid therapy did not achieve remission.

Clinical Scenario

The patient has EGPA without organ-threatening or life-threatening manifestations — a presentation in which treatment aims to achieve remission while matching therapy intensity to disease severity.

Previous Treatment: Glucocorticoids — Remission Not Achieved

The preceding treatment line used glucocorticoids with the goal of achieving remission, defined as the absence of typical signs, symptoms, or other features of active disease. Failure to reach this target is the trigger for escalation to this next-line protocol.

Next-Line Approach

The next-line protocol recommends mepolizumab as the primary treatment, with structured alternatives available for patients in whom it is not effective or not tolerated — targeting remission at 48 weeks. The full regimen, selection criteria, and complete sequence are in the protocol below.

References

DOI: 10.1136/ard-2022-223764

  • For induction of remission in new-onset or relapsing EGPA without organ-threatening or life-threatening manifestations, we recommend treatment with glucocorticoids.
  • For induction of remission in patients with relapsing or refractory EGPA without active organ-threatening or life-threatening disease, we recommend the use of mepolizumab.
  • Both co-primary endpoints (the number of weeks in remission on a prednisolone dose reduced to 4 mg and the proportion of patients in remission at weeks 36 and 48) were met in favour of mepolizumab.
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