Treatment of Eosinophilic Granulomatosis with Polyangiitis with Organ-Threatening or Life-Threatening Manifestations
Clinical Scenario
This protocol addresses patients with EGPA who present with severe disease involving one or more critical organ systems. Manifestations in scope include:
- Glomerulonephritis
- Pulmonary haemorrhage
- Cardiac involvement
- Central nervous system involvement
- Mononeuritis multiplex
- Mesenteric involvement
Treatment Approach
Induction of remission in this setting involves high-dose glucocorticoids combined with an immunosuppressive agent. An alternative combination regimen is also available for appropriate patients.
Agent selection, sequencing, tapering schedule, and the full regimen algorithm are in the complete protocol.
Goal: remission at 6 months
References
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.
- A combination of high-dose glucocorticoids and rituximab may be considered as an alternative.
- The primary endpoint of on-treatment remission was reached at similar frequencies at days 180 and 360 in both groups, but the limited number of patients, the superiority design and the lack of fully published results do not allow for strong conclusions regarding non-inferiority.
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