What is the treatment for Eosinophilic granulomatous polyangitis?

Treatment of Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Glucocorticoids are the cornerstone of EGPA treatment for all patients, with additional immunosuppressants (cyclophosphamide or rituximab) required for severe cases, while non-severe cases may be treated with glucocorticoids alone or in combination with mepolizumab. 1

Disease Severity Assessment

Disease severity determines treatment approach:

• Severe EGPA (any of the following):

  • Five-Factor Score (FFS) ≥1
  • Organ- or life-threatening manifestations (cardiomyopathy, gastrointestinal involvement, central nervous system involvement, alveolar hemorrhage)
  • Significant renal involvement
  • Severe peripheral neuropathy

• Non-severe EGPA:

  • Absence of the above features
  • Primarily respiratory/ENT manifestations

First-Line Treatment Algorithm

For Severe EGPA:

1. High-dose glucocorticoids:

   • Initial: IV pulse methylprednisolone (500-1000 mg/day for 3 days)
   • Followed by: Oral prednisone 1 mg/kg/day (maximum 60-80 mg/day)

2. PLUS one of the following:

   • Cyclophosphamide: IV pulses (15 mg/kg every 2-3 weeks) or oral (2 mg/kg/day)
   • Rituximab: 375 mg/m² weekly for 4 weeks 1, 2

For Non-Severe EGPA:

1. Glucocorticoids alone:

   • Oral prednisone 0.5-1 mg/kg/day

2. OR Glucocorticoids plus Mepolizumab:

   • Mepolizumab 300 mg subcutaneously every 4 weeks 1
   • Particularly useful for relapsing or refractory disease

Maintenance Therapy

After remission is achieved (typically 3-6 months):

1. Glucocorticoid tapering:

   • Aim for ≤7.5 mg prednisone daily
   • Target ≤4 mg daily when possible 1

2. Maintenance immunosuppression:

   • After cyclophosphamide induction: Switch to azathioprine, methotrexate, or leflunomide
   • After rituximab induction: Consider repeat rituximab dosing or switch to conventional immunosuppressants

Management of Specific Manifestations

Respiratory and ENT Involvement:

• Optimize topical/inhaled therapies
• Involve specialists (pulmonologists, otolaryngologists)
• Consider mepolizumab or benralizumab for predominant respiratory manifestations 1

Cardiac Involvement:

• Requires aggressive immunosuppression (cyclophosphamide or rituximab)
• Obtain echocardiogram at diagnosis and follow-up

Peripheral Neuropathy:

• May require more aggressive immunosuppression
• Consider electromyography-electroneurography for assessment 3

Monitoring and Follow-up

• Regular assessment of disease activity using validated tools (BVAS)
• Monitor blood counts, especially with cyclophosphamide or rituximab
• Assess organ-specific manifestations (lung function, cardiac status, neurological function)
• Monitor for treatment-related complications:
  • Infections (consider Pneumocystis jirovecii prophylaxis with trimethoprim-sulfamethoxazole for patients on cyclophosphamide or rituximab) 3
  • Osteoporosis (especially with prolonged glucocorticoid use)
  • Malignancy risk (particularly with cyclophosphamide)

Refractory Disease Management

For patients with inadequate response to first-line therapy:

1. Switch immunosuppressive strategy:

   • If on cyclophosphamide, consider rituximab
   • If on rituximab, consider cyclophosphamide

2. Consider biologic agents:

   • Mepolizumab (if not already used)
   • Benralizumab (30 mg subcutaneously every 4 weeks)
   • Tezepelumab (for refractory asthma component) 1

Special Considerations

• ANCA status: ANCA-positive patients (30-40%, mostly MPO-ANCA) more frequently show vasculitic features, while ANCA-negative patients more commonly have cardiomyopathy and lung involvement 1

• Vaccination: Complete age-appropriate vaccinations before initiating immunosuppression when possible

• Screening: Screen for chronic infections (hepatitis B, HIV) before starting cyclophosphamide or rituximab 3

Treatment Goals

• Control of vasculitis manifestations
• Asthma and ENT disease control
• Minimization of glucocorticoid exposure
• Prevention of organ damage and mortality
• Improvement in quality of life

The treatment of EGPA requires a multidisciplinary approach involving rheumatologists, pulmonologists, cardiologists, neurologists, and other specialists as needed based on organ involvement.