This protocol applies to patients with eosinophilic granulomatosis with polyangiitis presenting with organ-threatening or life-threatening disease activity — a high-stakes subset where preventing permanent organ damage is a primary clinical priority.
Management of this presentation involves a structured immunosuppressive strategy addressing both induction and maintenance of remission. Maintenance therapy draws on a defined set of agents — the complete protocol with full selection criteria and sequencing is accessible via the link below.
Sustained remission: absence of typical signs, symptoms, or other features of active vasculitis over a defined time period.
DOI: 10.1136/ard-2022-223764
For induction of remission in new-onset or relapsing EGPA with organ-threatening or life-threatening manifestations, we recommend treatment with a combination of high-dose glucocorticoids and cyclophosphamide.
With the aim of preventing permanent organ damage due to EGPA, patients with severe involvement of the kidneys, central and peripheral nervous system, or gastrointestinal tract are also considered to be candidates for treatment with CYC.
For maintenance of remission of EGPA after induction of remission for organ-threatening or life-threatening disease, treatment with either methotrexate, azathioprine, mepolizumab or rituximab should be considered.
Sustained remission: Absence of typical signs, symptoms, or other features of active AAV over a defined time period with or without immunosuppressive therapy.
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