A rare systemic vasculitis affecting less than two out of every million people each year, eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a condition that causes inflammation of small-sized blood vessels in people with asthma. About half of the cases of EGPA are also considered a systemic autoimmune disease affecting blood vessels: antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides.
Pulmonologists and thoracic surgeons who specialize in eosinophilic granulomatosis with polyangiitis at the Brigham and Women’s Hospital Lung Center provide comprehensive, specialized care for patients experiencing EGPA. Their expertise and collaboration with other specialists throughout the Brigham provide patients with a highly-informed diagnosis and a cohesive treatment plan.
What are the risk factors for EGPA?
While EGPA can occur at any age, the majority of patients are around age 50. The cause of EGPA is unknown. Potential risk factors may include:
- Asthma, allergic rhinitis and/or nasal polyps
- Drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers or mesalazine
- Environmental exposure to inhaled allergens such as silica dust
What are the symptoms of EGPA?
The symptoms of EGPA differ from person to person, depending on its severity and which organs are affected. The majority of patients have asthma, nasal polyps and/or blood eosinophilia. Other symptoms include:
- Fever
- Fatigue
- Rapid and unintentional weight loss
- Muscle and joint pain
- Skin rash, such as purpura (punctuated red lesions) skin nodules or recurrent hives
- Numbness, tingling or sudden loss of strength in the hands or feet
- Chest pain, palpitations or arrhythmias (irregular heartbeat)
- Shortness of breath or coughing
- Phlebitis (vein inflammation) or pulmonary embolism
- Abdominal pain
- Gastrointestinal bleeding
What are the stages of EGPA?
The disease typically manifests in three stages, although not all patients experience all three stages or progress in the same order. In the prodromal phase, patients have asthma, allergic rhinitis and/or nasal polyposis. The second stage is characterized by abnormally-high numbers of eosinophils (a type of white blood cell) that cause tissue damage to the lungs and digestive tract. The third phase involves inflammation of the blood vessels that reduces blood flow to various organs and tissues. Cardiac-related complications are frequent, and as a result EGPA can be fatal if left untreated.
How is EGPA diagnosed?
Your physician will diagnose EGPA based on a careful review your medical history, a full physical examination and additional test results, including:
What is the treatment for EGPA?
Eosinophilic Granulomatosis with Polyangiitis is treated with medications. Less severe cases of EGPA can often be treated with corticosteroids alone, while patients with severe cardiac, cerebral, gastrointestinal or renal side effects usually require a combination of therapies, including:
- Corticosteroids, mainly prednisone
- Immunosuppressant drugs, such as azathioprine, cyclophosphamide, methotrexate or rituximab
- Intravenous steroids, usually methylprednisolone
Patients require continuous follow-up to ensure that relapse or other asthma-related complications do not occur.
What can you expect for EGPA care?
A multidisciplinary team will work with you every step of the way, from diagnostics through evaluation, to create a cohesive and comprehensive treatment plan. Our unique approach features same day consultations with multiple specialists and fosters seamless, expert care. Your medical condition will be closely monitored and managed to promote optimal lung functioning and an improved quality of life.
Who treats EGPA?
EGPA patients benefit from the wide range of specialists at The Lung Center, including thoracic surgeons, pulmonologists and imaging experts. This collaboration ensures comprehensive diagnosis and targeted treatment for patients.
How do you make an appointment of find directions?
What are other resources are available on EGPA?
Learn more about topics related to Eosinophilic Granulomatosis with Polyangiitis in our health library.
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