Churg-Strauss Syndrome (EGPA): The Complete Guide to Symptoms, Stages, and Treatment
Updated: 3 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick Answer: What Is Churg-Strauss Syndrome (EGPA)?
Churg-Strauss syndrome — now called eosinophilic granulomatosis with polyangiitis (EGPA) — is a rare condition that causes inflammation in small-to-medium-size blood vessels, reducing blood flow to organs such as the heart, kidneys, and nerves. The first sign is typically asthma that develops in adulthood. Although there is no cure, treatment with steroids and other immune-calming medicines can manage symptoms. [1]
What the research says
Heart involvement is the leading cause of death, and many patients test negative for ANCA. A clinical review of EGPA notes that fewer than 50% of patients have a positive ANCA test, that polyneuropathies are common and can be severe, and that heart involvement is the most common cause of death; relapses are common and prolonged corticosteroid treatment is often necessary. (Villa-Forte, Postgrad Med, 2023) [3]
Anti-IL-5 medicines can let some patients stop steroids entirely. In a post hoc analysis of the MANDARA trial, 140 adults with relapsing or refractory EGPA received benralizumab 30 mg (n = 70) or mepolizumab 300 mg (n = 70) every 4 weeks for 52 weeks; the adjusted share in remission off oral glucocorticoids with no relapses was 23.5% with benralizumab versus 11.1% with mepolizumab (P = 0.042). (Wechsler et al., ACR Open Rheumatol, 2025) [4]
Rituximab has not clearly beaten conventional treatment. A systematic review found one randomized trial of 105 patients in which 180-day remission was 63.5% with rituximab versus 60.4% with conventional therapy (risk ratio 1.05, 95% CI 0.78–1.42; very low certainty), while benralizumab and mepolizumab produced similar remission rates (58.6% versus 55.7%). (Shiomi et al., Mod Rheumatol, 2026) [5]
Churg-Strauss Syndrome (EGPA) at a Glance
Aspect | Summary |
Also known as | EGPA — eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) |
What it is | A rare condition causing inflammation in small-to-medium-size blood vessels |
First sign | Asthma that develops in adulthood; nasal allergies, sinus infections, and nasal polyps are common |
Typical diagnosis age | Around 50, though anyone can develop it |
Key organs at risk | Heart, kidneys, nerves, skin, lungs, sinuses, digestive system, muscles, joints |
Diagnosis | No single test — blood tests, imaging, and tissue biopsy combined |
Main treatment | Corticosteroids (prednisone), plus mepolizumab and benralizumab — the only FDA-approved medicines for EGPA |
Outlook | No cure, but treatment manages symptoms, prevents flare-ups, and protects organs |
What Is Churg-Strauss Syndrome (EGPA)?
Eosinophilic granulomatosis with polyangiitis, shortened to EGPA, is a rare condition that causes inflammation in small- to medium-size blood vessels. This inflammation can lessen blood flow to important organs and tissues, such as the heart, kidneys, and nerves. If not treated, this condition can damage organs and tissues.
EGPA used to be called Churg-Strauss syndrome — you will see both names used, and they refer to the same disease.
The first sign of EGPA is typically asthma that develops in adulthood. Many people also have nasal allergies, sinus infections, and nasal polyps. As EGPA progresses, it may cause rashes, stomach pain or bleeding, and numbness or tingling in the hands and feet.
Although there is no cure, treatment with steroids and other medicines that calm the immune system can help manage symptoms and lessen complications. These treatments depend on how serious the condition is and which organs are affected.
What are the three stages of Churg-Strauss syndrome (EGPA)?
EGPA symptoms are different for each person. Some people have mild symptoms, while others may develop serious or even life-threatening symptoms. Without treatment, symptoms typically get worse over time.
The condition often progresses through three stages — which may overlap, and not everyone goes through all three:
How EGPA typically progresses through its three stages. Source: rinnit.com
Stage 1: The Allergic Stage
This first stage often lasts for years. It typically includes new or worsening asthma, nasal allergies, and repeated sinus infections.
Stage 2: The Eosinophilic Stage
In this stage, the body makes too many eosinophils. Eosinophils are a type of white blood cell that plays a role in allergic reactions. When they build up in tissues and organs, they cause damage. Common issues in this stage include:
Lung symptoms such as coughing or wheezing
Digestive issues such as stomach pain or bleeding, nausea, and vomiting
Stage 3: The Vasculitic Stage
This final stage involves vasculitis — the medical term for inflammation of small- to medium-size blood vessels. Vasculitis lessens blood flow and may harm the heart, kidneys, skin, nerves, and other organs.
What are the symptoms of Churg-Strauss syndrome (EGPA)?
Beyond stage-specific signs, EGPA symptoms may include weight loss, joint and muscle pain, fatigue, skin rash, numbness or tingling in the hands and feet, nasal polyps and hearing loss.
Loss of appetite and weight loss
Joint and muscle pain
Fatigue, weakness, or a general feeling of being unwell
Skin rash or sores
Pain, numbness, or tingling in the hands and feet
Nasal polyps
Hearing loss
When should you see a doctor about Churg-Strauss syndrome (EGPA)?
See your healthcare professional if you have asthma or nasal allergies that suddenly get worse. See your healthcare professional if you develop breathing difficulties or a runny nose that doesn't go away, especially if you have facial pain as well. And see your healthcare professional if you have unusual rashes, digestive issues, or burning, numbness, or tingling in your hands and feet.
These symptoms can be caused by many conditions. But if EGPA is suspected, early diagnosis and treatment are key to preventing serious complications.
Causes: Why Does EGPA Happen?
The exact cause of EGPA isn't fully known. It's likely a combination of genetic and environmental factors. For some people, things such as allergens or certain medicines may trigger an overreaction in the immune system.
And instead of protecting the body from bacteria and viruses, the immune system starts attacking healthy tissues. Over time, this can lead to inflammation in the blood vessels and damage to organs and tissues.
What are the risk factors for Churg-Strauss syndrome (EGPA)?
Anyone can develop EGPA, but it most often is diagnosed in people around age 50. You may be at risk if you have:
A history of asthma, especially asthma that started when you were an adult
Ongoing nasal or sinus issues
Allergies
Nasal polyps
A family history of autoimmune diseases
Contact with things that may trigger allergies or immune responses
Having these risk factors doesn't mean you'll develop EGPA, but they may increase your chances.
What complications can untreated Churg-Strauss syndrome (EGPA) cause?
EGPA can affect many parts of the body, including the lungs, sinuses, skin, digestive system, kidneys, muscles, joints and heart. If not treated, the condition can lead to permanent damage and may be life-threatening. Complications depend on the organs involved:
Complication | What happens |
Nerve damage (neuropathy) | EGPA can damage the nerves in your hands and feet, causing numbness, burning, pain, and muscle weakness |
Heart disease | Inflammation can affect the heart and lead to chest pain, heart attack, or heart failure |
Kidney damage | EGPA can cause glomerulonephritis, a condition that lessens your kidneys' ability to filter waste, leading to serious health issues |
How is Churg-Strauss syndrome (EGPA) diagnosed?
There is no single test to diagnose EGPA. Healthcare professionals typically use a combination of tests to look for signs of inflammation, high eosinophil levels, and damage to organs. [2]
Test | What it looks for |
Blood tests | Certain antibodies in your blood can suggest (but not confirm) a diagnosis; high eosinophil levels can support the diagnosis but are not specific — other conditions, including asthma, also increase eosinophils |
Imaging tests | X-rays, MRI scans, or CT scans can show issues in the lungs or sinuses; if heart issues are suspected, an echocardiogram looks at how your heart is working |
Tissue biopsy | A small sample of tissue may be taken from your lungs, skin, or another affected area and examined under a microscope for inflamed blood vessels (vasculitis) to confirm the diagnosis |
Your care team also considers your medical history, symptoms, and response to treatment when making a diagnosis.
How is Churg-Strauss syndrome (EGPA) treated with steroids and immune-calming medicines?
Although there's no cure for EGPA, medicines can help reduce inflammation, manage symptoms, and prevent complications.
Corticosteroids: The Main Treatment
The main treatment for EGPA is a steroid called prednisone. This medicine helps lessen inflammation quickly. You may start on a high dose to get the condition under control, then your healthcare professional gradually lowers the dose to the smallest amount needed to keep your symptoms under control.
Taking corticosteroids at high doses or for a long time can lead to serious side effects, including:
Weight gain
High blood sugar or diabetes
Cataracts
Infections that are hard to treat
Bone loss (osteoporosis)
Your healthcare team will monitor you closely and may recommend steps to reduce these risks.
Other Immune-Calming Medicines
For people with mild symptoms, a corticosteroid alone may be enough. Other people may need more than just steroids. Your healthcare professional may add other medicines to help calm the immune system, especially if your symptoms are more serious or if steroids alone aren't enough.
The only medicines approved by the U.S. Food and Drug Administration specifically for EGPA are:
Medicine | Brand name | What it does |
Mepolizumab | Nucala | Targets eosinophils; often used with other treatments |
Benralizumab | Fasenra | Targets eosinophils; often used with other treatments |
Other medicines that may be used include:
Medicine | Brand names |
Azathioprine | Azasan, Imuran |
Cyclophosphamide | Cytoxan |
Methotrexate | Trexall |
Rituximab | Rituxan |
These medicines can weaken your immune system, so your healthcare team monitors you for infections and other side effects. The goal is to control the inflammation, prevent flare-ups, and protect your organs.
How can you protect your health while taking steroids for EGPA?
Long-term corticosteroid use for EGPA can cause side effects such as bone loss and high blood sugar. You can lower your risk by taking good care of your overall health:
Protect your bones. Ask your healthcare professional how much vitamin D and calcium you should get in your diet. Talk with your healthcare professional about whether you should take supplements to help prevent bone loss.
Stay active. Exercise helps keep your bones strong and your weight in a healthy range. Activities such as walking, jogging, and strength training are especially helpful to improve bone health.
Eat a healthy diet. Steroids can cause high blood sugar levels and, eventually, type 2 diabetes. A healthy diet can help manage your weight and keep blood sugar levels steady. Choose foods such as fruits, vegetables, lean proteins, and whole grains.
How can people cope with Churg-Strauss syndrome (EGPA)?
Living with an illness like EGPA can be challenging, but learning about the condition and building a support system may help. These tips may help you cope:
Learn about the condition. Understanding EGPA helps you manage your care and prepare for changes.
Build a support system. Family and friends can be a great source of comfort and support. You also may benefit from talking to a counselor or medical social worker who's familiar with the disease. Many people find it helpful to join a support group for people living with EGPA. Talk with members of your care team about online or in-person support groups they may recommend.
How should you prepare for an appointment about Churg-Strauss syndrome (EGPA)?
If you have symptoms of EGPA, make an appointment with your healthcare professional. Early diagnosis and treatment can improve your outcome. You may be referred to a specialist, such as a:
Rheumatologist — a doctor who specializes in joint and immune system diseases
Immunologist — a doctor who specializes in immune system diseases
Pulmonologist — a doctor who specializes in lung conditions
What You Can Do
Ask about pre-appointment requirements — such as avoiding food or drink, or staying for observation after any tests.
Bring a list of your symptoms and when they started, even those that seem unrelated to EGPA.
Bring your key medical information, including other conditions with which you've been diagnosed.
List all medicines, vitamins, and supplements you take, including doses.
Bring notes or letters summarizing findings from other healthcare professionals you've seen, and copies of recent chest or sinus X-rays if you have them.
Take a family member or friend along to help you remember all of the information you receive.
Write down questions to ask your healthcare professional.
Basic Questions to Ask
What do you think is causing my symptoms?
Are there other possible causes?
What tests will I need?
What treatment do you recommend?
What healthy changes can I make to help reduce or manage my symptoms?
How often should I come in for follow-up tests?
What to Expect from Your Doctor
Your healthcare professional is likely to ask you:
When did your symptoms start?
Have your asthma or allergy symptoms gotten worse?
Do you have breathing issues or sinus infections?
Do you have stomach issues such as nausea or diarrhea?
Have you noticed numbness, pain, or weakness in your arms or legs?
Have you lost weight without trying?
Do you have any history of allergies, asthma, or autoimmune conditions?
Frequently Asked Questions (FAQ)
What is Churg-Strauss syndrome?
Churg-Strauss syndrome is now called eosinophilic granulomatosis with polyangiitis (EGPA). It is a rare condition that causes inflammation in small-to-medium-size blood vessels, reducing blood flow to organs like the heart, kidneys, and nerves. It used to go by the name Churg-Strauss syndrome.
What causes Churg-Strauss syndrome?
The exact cause isn't fully known, but it is likely a combination of genetic and environmental factors. Allergens or certain medicines may trigger an immune system overreaction that attacks healthy tissues instead of protecting the body.
What are the three stages of EGPA?
EGPA often progresses through an allergic stage (new or worsening adult-onset asthma, nasal allergies, repeated sinus infections), an eosinophilic stage (excess eosinophil white blood cells build up in tissues), and a vasculitic stage (inflammation of blood vessels that can harm the heart, kidneys, skin, and nerves). Stages may overlap, and not everyone goes through all three.
How is EGPA diagnosed?
There is no single test. Doctors combine blood tests (looking for antibodies and high eosinophils), imaging tests (X-rays, MRI, CT, echocardiogram), and a tissue biopsy examined under a microscope for inflamed blood vessels — along with your medical history and symptoms.
Is Churg-Strauss syndrome curable?
No — there is currently no cure for EGPA. However, treatment with steroids and other immune-calming medicines can manage symptoms, prevent flare-ups, and lessen complications.
What is the main treatment for EGPA?
The main treatment is a corticosteroid called prednisone, started at a high dose and gradually reduced. The only medicines FDA-approved specifically for EGPA are mepolizumab (Nucala) and benralizumab (Fasenra), both of which target eosinophils; other immune-calming medicines such as azathioprine, methotrexate, cyclophosphamide, and rituximab may also be used.
Who is most likely to get EGPA?
Anyone can develop EGPA, but it is most often diagnosed around age 50. People with asthma, especially asthma that started in adulthood, ongoing sinus problems, allergies, or nasal polyps are at higher risk.
What are the symptoms of EGPA?
Early signs often include worsening asthma, nasal allergies, and sinus problems. Later signs can include rashes, digestive problems, and numbness, burning, or tingling in the hands and feet.
Can EGPA affect the heart?
Yes. Inflammation can affect the heart and lead to chest pain, heart attack, or heart failure. Call 911 for chest pain or sudden shortness of breath.
Is Churg-Strauss syndrome contagious?
No. EGPA is an immune system condition, not an infection, so it cannot spread from person to person.
Conclusion
Churg-Strauss syndrome — EGPA — is a rare disease that hides behind familiar symptoms. Three lessons stand out from this guide: adult-onset asthma is the signature clue — when asthma suddenly appears or worsens in adulthood, especially with sinus infections and nasal polyps, it can be the first signal of something deeper than allergies; the disease unfolds in stages — understanding the allergic, eosinophilic, and vasculitic stages helps you recognize where the damage can strike: lungs, digestion, nerves, heart, and kidneys; and treatment protects what matters most — there is no cure, but prednisone, FDA-approved eosinophil-targeting medicines, and careful self-care can control inflammation, prevent flare-ups, and protect your organs.
This guide covered the full journey — from early warning signs through diagnosis to treatment and self-care — so you can spot the pattern early and advocate for yourself with your care team.
What to do next: If you want to explore how the immune system can turn on the body in other ways, read our guides on autoimmune epilepsy, where the immune system attacks the brain, and Guillain-Barré syndrome, which, like EGPA, can damage the nerves in your hands and feet.
This content is provided for general informational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of a qualified healthcare provider with any questions about a medical condition.
Related Reading
ANCA-Associated Vasculitis (AAV): What It Is, Why Blood Vessels Become Inflamed, and How Treatment Prevents Organ Damage explains the vasculitis group that includes EGPA.
Granulomatosis with Polyangiitis (GPA): Symptoms, Causes, Diagnosis, and Treatment Explained compares a related vasculitis that affects the sinuses, lungs, and kidneys.
Eosinophilia: Symptoms, Causes, Related Disorders, Diagnosis, Treatment and When to See Your Provider — What You Need to Know explains high eosinophil counts, a hallmark of EGPA.
Vasculitis: Symptoms, Causes, Treatment & Complete Guide covers blood vessel inflammation types, symptoms, and treatment.
References
Mayo Clinic Staff. EGPA (formerly Churg-Strauss syndrome) — Symptoms and causes. Mayo Clinic. September 15, 2025. https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/symptoms-causes/syc-20353760
Mayo Clinic Staff. EGPA (formerly Churg-Strauss syndrome) — Diagnosis and treatment. Mayo Clinic. September 15, 2025. https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
Villa-Forte A. Eosinophilic granulomatosis with polyangiitis. Postgrad Med. 2023;135(sup1):52-60. PMID 36259957. https://doi.org/10.1080/00325481.2022.2134624
Wechsler ME, Agmon-Levin N, Jayne DRW, et al. Discontinuation of oral glucocorticoids and achievement of remission in patients with eosinophilic granulomatosis with polyangiitis treated with benralizumab or mepolizumab. ACR Open Rheumatol. 2025;7(9):e70096. PMID 40886121. https://doi.org/10.1002/acr2.70096
Shiomi M, Higuchi T, Akiyama M, et al. Updated treatment approaches for eosinophilic granulomatosis with polyangiitis: a systematic scoping review. Mod Rheumatol. 2026;36(3):415-421. PMID 41427848. https://doi.org/10.1093/mr/roaf126
Medical disclaimer
The information provided in this article is for educational purposes only and should not be used as a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
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