About Eosinophilic Granulomatosis with Polyangiitis
EGPA is classified as an ANCA-associated vasculitis alongside granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), though it is distinguished by its eosinophil-driven pathology and near-universal association with asthma. The disease involves both eosinophil-mediated tissue damage (through release of major basic protein, eosinophil cationic protein, and other toxic granule contents) and vasculitis (inflammation and necrosis of blood vessel walls).
Clinically, EGPA is divided into two phenotypes. The ANCA-positive subset (about 30 to 40% of patients) tends to have more renal, neurological, and purpura involvement. The ANCA-negative subset is associated with more cardiac and pulmonary infiltrative disease. Cardiac involvement, present in 15 to 60% of patients depending on the screening method, is the primary driver of mortality and can manifest as eosinophilic myocarditis, coronary arteritis, or heart failure.
Two anti-IL-5 therapies are now FDA-approved for EGPA. Mepolizumab (Nucala) was first, approved in December 2017 based on the MIRRA trial. In September 2024, benralizumab (Fasenra), an anti-IL-5 receptor alpha antibody, became the second approved therapy. In the Phase 3 MANDARA trial, which compared benralizumab head-to-head against mepolizumab, benralizumab achieved remission in nearly 60% of patients and allowed 41% to fully taper off oral corticosteroids.
Common Symptoms of Eosinophilic Granulomatosis with Polyangiitis
Recognizing the signs of Eosinophilic Granulomatosis with Polyangiitis early can lead to faster diagnosis and better outcomes. Symptoms may vary in severity from person to person. If you or a loved one are experiencing any of the following, consider speaking with a specialist.
- Severe adult-onset asthma that is difficult to control
- Chronic sinusitis and nasal polyps
- Peripheral neuropathy (numbness, tingling, weakness in hands or feet)
- Skin findings including purpura, nodules, and urticarial rash
- Heart problems including myocarditis, pericarditis, and heart failure
- Elevated blood eosinophil counts (often >1,500 cells/microL)
Who Eosinophilic Granulomatosis with Polyangiitis Affects
Mean age of onset is approximately 50 years. Affects males and females roughly equally across all ethnicities.
Almost all patients have a history of asthma (often severe) and allergic rhinitis years before vasculitic symptoms develop. ANCA antibodies (anti-MPO) are present in about 30 to 40% of patients and correlate with renal and neurological involvement.
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FDA-Approved Treatments for Eosinophilic Granulomatosis with Polyangiitis
There are currently 2 FDA-approved medications for Eosinophilic Granulomatosis with Polyangiitis. These therapies represent the current standard of care and may be used alongside or compared against investigational treatments in active clinical trials.
Source: openFDA drug labeling data. This list may not include all treatments. Always consult your doctor.
Help Paying for Eosinophilic Granulomatosis with Polyangiitis Treatment
Charity funds and drugmaker programs for Eosinophilic Granulomatosis with Polyangiitis, checked at the source. Pick your insurance to see what fits.
- From a charity · HealthWell FoundationANCA-Associated Vasculitis and Granulomatosis with Polyangiitis fundOpen
Pays for: Copays, premiums or other treatment costs.
Side Effect Explorer
Real-world side effect reports from the FDA Adverse Event Reporting System (FAERS). Includes both FDA-approved drugs and investigational therapies from active clinical trials. Click any drug to see what patients reported.
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Genetic Testing
Genetic testing can confirm a diagnosis, guide treatment decisions, and identify family members who may be at risk.
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Trusted Eosinophilic Granulomatosis with Polyangiitis Resources
Reputable organizations and medical references for learning more about Eosinophilic Granulomatosis with Polyangiitis, including disease registries, foundation resources, and clinical guidelines.
