From the Guidelines
Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a complex condition that requires a tailored treatment approach, and the most recent evidence suggests that for remission induction in patients with new-onset, active EGPA, glucocorticoids should be administered as initial therapy, with cyclophosphamide or rituximab added in cases of severe disease 1.
Symptoms of EGPA
The symptoms of EGPA can vary widely and may include:
- Asthma that becomes difficult to control
- Sinusitis
- Skin rashes or purpura
- Numbness or tingling in extremities due to peripheral neuropathy
- Abdominal pain
- Fatigue
- Muscle and joint pain
- Cough
- Shortness of breath
- Heart complications like myocarditis or heart failure in severe cases
Treatment Options for EGPA
Treatment for EGPA typically involves a two-phase approach:
- For remission induction in severe disease, high-dose corticosteroids (such as prednisone 1 mg/kg/day, maximum 60-80 mg daily) are combined with immunosuppressants like cyclophosphamide (15 mg/kg IV every 2-3 weeks) or rituximab (375 mg/m² weekly for 4 weeks) 1.
- For milder cases, corticosteroids alone may suffice.
- Once remission is achieved, maintenance therapy follows with lower-dose corticosteroids gradually tapered over months, plus azathioprine (2 mg/kg/day), methotrexate (15-25 mg weekly), or mycophenolate mofetil (1-2 g daily) 1.
- Mepolizumab, an anti-IL-5 monoclonal antibody, has been approved specifically for EGPA at 300 mg subcutaneously every 4 weeks and can be considered for remission induction in non-severe EGPA, especially in patients with a history of relapsing or refractory disease 1.
Key Considerations
- Regular monitoring of blood counts, inflammatory markers, and organ function is essential during treatment 1.
- The treatment approach should be individualized based on disease severity, patient factors, and response to therapy 1.
- Collaboration between rheumatologists, asthma/allergy specialists, and other medical disciplines can enhance patient care 1.
From the Research
Symptoms of Eosinophilic Granulomatosis with Polyangiitis (EGPA)
- The most common onset symptom of EGPA is asthma, which is present in approximately 70% of patients 2
- Other common symptoms include sinusitis, rhinitis, and peripheral neuropathy, which are present in 93%, 93%, and 60.5% of patients, respectively 2
- Patients with EGPA may also experience fever, renal disease, and heart involvement, which can be severe and require aggressive treatment 3, 4
- The disease can affect multiple organ systems, and the clinical manifestations may vary widely 2, 5
Treatment Options for EGPA
- Treatment of EGPA is primarily dictated by the severity of disease and prognostic factors 3
- Glucocorticoid (GC) therapy is often initiated before a definitive diagnosis is made, and immunosuppressive agents may be added in more severe cases 5
- Anti-interleukin (IL)-5/IL-5 receptor alpha (IL-5Rα) antibodies targeting eosinophils have become increasingly important in the treatment of EGPA, and may have effects beyond those observed in eosinophils 5
- Cyclophosphamide may be used in more severe cases, and prolonged treatment with corticosteroids is often necessary for asthma management 3
- Monitoring of the fractional exhaled nitric oxide (FeNO) level, eosinophil percentage and count, and total immunoglobulin E (IgE) level may be useful in diagnosing and managing EGPA 4
Diagnosis of EGPA
- A biopsy of an injured organ can be useful for diagnosis, but is not always feasible due to its invasiveness and diagnostic accuracy 5
- A comprehensive diagnosis is often made based on symptoms and clinical course of disease, and may involve the use of classification criteria such as the DCVAS diagnostic criteria 6
- Cardiac emission computed tomography (ECT) tests may be useful in detecting cardiac involvement, which is a common cause of death in EGPA 4