From the Guidelines
Treatment for Eosinophilic Granulomatosis with Polyangiitis (EGPA) should be tailored to disease severity, with more aggressive therapy for patients with critical organ involvement and milder approaches for those with primarily respiratory symptoms, as recommended by the 2023 evidence-based guideline for the diagnosis and management of EGPA 1.
Key Considerations
- For patients with new-onset, active EGPA, glucocorticoids should be administered as initial therapy, with cyclophosphamide or rituximab added for severe disease 1.
- For remission maintenance, rituximab, mepolizumab, or traditional DMARDs in combination with glucocorticoids are recommended for patients with severe EGPA, while glucocorticoids alone or in combination with mepolizumab are suggested for patients with non-severe EGPA 1.
- The IL-5 inhibitor mepolizumab in combination with glucocorticoids is recommended to induce remission in patients with relapsing-refractory EGPA without organ- or life-threatening manifestations 1.
Treatment Options
- Glucocorticoids: high-dose prednisone (1 mg/kg/day, maximum 60-80 mg daily) to rapidly control inflammation, with gradual tapering over several months 1.
- Cyclophosphamide: 2 mg/kg/day orally or 15 mg/kg IV every 2-3 weeks for 3-6 months for severe or organ-threatening disease 1.
- Rituximab: 375 mg/m² weekly for 4 weeks for refractory cases or to reduce steroid dependence 1.
- Mepolizumab: 300 mg subcutaneously every 4 weeks, specifically approved for EGPA and targeting the eosinophilic component of the disease 1.
Monitoring and Adjustment
- Regular monitoring of blood counts, organ function, and disease activity is essential to adjust therapy and minimize side effects 1.
- Treatment decisions should be modified as necessary in special populations of patients, such as children, elderly patients, women of child-bearing age, and those with comorbidities 1.
From the Research
EGPA Treatment Overview
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of small-vessel vasculitis characterized by asthma, hyper-eosinophilia, and progressive multiorgan involvement 2.
- Traditional treatment for EGPA involves glucocorticoids, either alone or in combination with conventional immunosuppressants 3.
Biologic Therapies for EGPA
- Mepolizumab, an anti-interleukin (IL)-5 agent, has been approved as an add-on therapy for adult patients with EGPA and is effective in induction and maintenance of remission, particularly in patients with predominantly asthma and allergic manifestations 2, 4.
- Rituximab, an anti-CD20 monoclonal antibody, is recommended for remission induction in severe disease, particularly in ANCA-positive patients with vasculitic manifestations 5, 4.
- Other biologic agents, such as omalizumab, are being studied, but evidence supporting their use remains limited and observational 4.
Treatment Strategies and Outcomes
- A lower-than-recommended dose of mepolizumab (100 mg SC, every 4 weeks) has been shown to be effective in achieving reasonable clinical efficacy in the long-term treatment of EGPA in some patients, enabling the reduction of glucocorticoid doses 2.
- Rituximab treatment has been associated with successful reduction of glucocorticoid dose and induction of remission in patients with newly diagnosed or relapsing and refractory EGPA, with potentially greater benefit in ANCA-positive patients than in ANCA-negative patients 5.
- Scheduled rituximab maintenance treatment has been shown to significantly reduce relapse rates compared to rituximab administered on demand 5.