Eosinophilic Granulomatosis With Polyangiitis: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Eosinophilic Granulomatosis With Polyangiitis?
A rheumatologist (a doctor who treats immune system and blood vessel diseases) usually leads care for eosinophilic granulomatosis with polyangiitis. A pulmonologist (a lung doctor) or an allergist (a doctor who treats allergies and asthma) often helps too. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is eosinophilic granulomatosis with polyangiitis?
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare immune disorder of the blood vessels. It can affect many organs, especially the lungs. It was once called Churg-Strauss syndrome.
People with EGPA have many eosinophils, a type of white blood cell, in the blood and tissues. This causes vasculitis, which means inflamed blood vessels. It can also cause granulomas, which are inflamed lumps in tissue.
When blood vessels are inflamed, they can narrow, close off, or stretch and bulge.
Symptoms
Symptoms can vary a lot, because different organs can be affected in each person.
Many people first have nose and sinus symptoms that look like allergies or a sinus infection. Worsening asthma often comes before more serious signs. Most people with EGPA develop asthma.
Signs can include:
- General signs: fever, tiredness, weight loss, loss of appetite, and joint and muscle pain.
- Lungs: cough, wheezing, and trouble breathing.
- Nose: nasal polyps, which are inflamed growths inside the nose.
- Belly: pain, nausea, and vomiting.
- Nerves: numbness or tingling in the arms or legs.
- Heart: tiredness, shortness of breath, chest pain, and fainting.
Kidney problems are rare but can be severe.
If EGPA is not treated, it can lead to serious problems. These include permanent nerve damage and stroke. They also include heart failure, irregular heart rhythms, and heart attacks.
Causes and who gets it
Doctors do not know the exact cause of EGPA. Researchers think that several factors play a role, including the environment, the immune system, and genes.
Researchers also think there may be two types of EGPA. In one type, EGPA is classified as an autoimmune disorder.
In this type, a protein called ANCA is sometimes present. It is found in 30 to 50 percent of people with EGPA, depending on the study. Kidney problems and bleeding in the lungs are more common in these people.
EGPA happens equally in men and women. It can affect almost any age, but it is rarer in children, teens, and older adults. Most people are diagnosed between ages 38 and 54.
EGPA may instead be linked to stopping medicines that control allergic or eosinophil inflammation, such as steroids. People who take asthma medicines should lower them only with monitoring, after steroid-sparing treatments are started.
How doctors diagnose it
Doctors may suspect EGPA after a careful exam and special tests, especially in people with asthma, sinus problems, and a high eosinophil count in the blood.
Blood tests can show high levels of eosinophils. They can also show high levels of IgE, an antibody involved in allergies.
Chest x-rays and CT scans, which are detailed x-ray pictures, can show changes in the sinuses and lungs.
A biopsy removes a small piece of tissue, such as from the skin, a nerve, the kidney, or the lung. A doctor then looks at it under a microscope for signs of inflamed blood vessels.
Other conditions can look like EGPA, so doctors keep them in mind.
Treatment
EGPA can be managed well with the right treatment.
Treatment aims to put people in remission, which means the disease is under control.
Corticosteroids, which are steroid medicines such as prednisone, are usually the first treatment. Doctors start with a large amount and then lower it when the person improves. For many people with non-severe EGPA, steroids alone may be enough to reach remission.
Other medicines can help prevent the harmful effects of using large amounts of steroids for a long time.
For severe EGPA, doctors use steroids through a vein, then steroid pills with cyclophosphamide or rituximab. These medicines calm the immune system. After remission, steroids may be combined with rituximab, mepolizumab, or DMARDs (disease-modifying antirheumatic drugs) to help keep the disease in remission and lower the chance of relapse.
Mepolizumab (Nucala) is recommended to help people with non-severe EGPA stay in remission.
In 2024, the FDA approved benralizumab (Fasenra) for adults with EGPA. It helps people reach remission and lower their steroid use.
When to see a doctor
Late-onset asthma and allergy symptoms are often the earliest signs of EGPA. Tell your doctor about them, especially if you also have fever, weight loss, or numbness and tingling.
Call 911 or go to the emergency room if you have chest pain or discomfort lasting two minutes or more, severe trouble breathing, coughing up blood, fainting, or sudden weakness or a change in vision.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Such a doctor can review your symptoms, order first tests, and help coordinate care with specialists such as a rheumatologist.
Common questions
Is Churg-Strauss syndrome the same as EGPA?
Yes, Churg-Strauss syndrome is the older name for EGPA.
Is EGPA an autoimmune disease?
Yes, in some people. For most people with EGPA, an autoantibody such as ANCA is not found.
Can EGPA be treated?
Yes, EGPA can be managed well with the right treatment.
How common is EGPA?
EGPA is rare. One estimate in the USA is 3 to 30.7 people per million, among people with insurance.
Sources
- National Organization for Rare Disorders (NORD) - Eosinophilic Granulomatosis with Polyangiitis
- MedlinePlus - Vasculitis
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
