Eosinophilic granulomatosis with polyangiitis

Overview

Eosinophilic granulomatosis with polyangiitis (EGPA), also called Churg-Strauss syndrome, is a very rare condition in which the body’s immune system mistakenly attacks small and medium-sized blood vessels. This causes inflammation that can damage organs such as the lungs, skin, nerves, and sometimes the heart or kidneys. It is closely linked to asthma and high levels of a type of white blood cell called eosinophils.

EGPA is very rare. It affects only a small number of people in every million each year, so most doctors will see only a few cases in their career.

EGPA can occur at any age, but it most often starts in people between 40 and 60. People who have asthma or allergic sinusitis are more likely to develop it, but the vast majority of people with asthma will never get this condition.

Symptoms

Common symptoms

• New or worsening asthma, often with wheezing or coughing
• Sinus pain, a blocked or runny nose, and nasal polyps (small growths inside the nose)
• Numbness, tingling, or burning pain in the hands or feet (a sign of nerve involvement)
• Joint or muscle aches and pains
• Skin rashes, bumps, or sores, including reddish-purple patches
• Constant fatigue, low-grade fever, night sweats, and unintended weight loss
• High levels of eosinophils (a type of white blood cell) found on a blood test

Causes

Main causes

• The exact cause is not known.
• Doctors think EGPA is an autoimmune response, meaning the immune system mistakenly attacks healthy blood vessels.
• A mix of genetic and environmental factors — such as an infection or an allergen — may trigger the disease in someone who is already prone to it.

Risk factors

• Having asthma, especially difficult-to-control asthma, commonly over many years
• Having allergic sinusitis or nasal polyps
• Being middle-aged, especially between 40 and 60
• Being male or female (the condition can affect either, though some studies suggest it is slightly more common in men)

When to see a doctor

See a doctor urgently if:

• If you or someone else has sudden chest pain, coughing blood, fainting, or stroke-like symptoms, call your local emergency number right away.
• If you have a severe headache with a high fever, new confusion, or sudden weakness, get urgent same-day medical care.

Book a routine appointment if:

• Make an appointment if your asthma is getting harder to control.
• See a doctor if you have ongoing sinus symptoms, nasal polyps, or unusual tingling or numbness in your hands or feet.
• If you have a rash that is spreading, fatigue that will not go away, or unexplained joint/muscle pain, discuss it with a healthcare provider.

Diagnosis

There is no single test for EGPA. A doctor will begin by listening to your medical history, especially any history of asthma or sinus problems, then examine you and order blood tests. If EGPA is suspected, you will usually be referred to a specialist, such as a rheumatologist, immunologist, or other specialist depending on which organs are affected.

Tests that may be done

• Blood tests — including a complete blood count to check white blood cells and eosinophils, and tests that look for inflammation (such as C-reactive protein and ESR)
• Tests for specific immune system markers, like ANCA, which can appear in some autoimmune conditions
• Chest X-ray or CT scan to look at the lungs and airways
• A biopsy — a small sample of tissue taken from an affected area, like skin or sinus tissue — to look for inflamed blood vessels
• Nerve conduction studies if you have tingling or numbness in the hands or feet
• Urine tests to check whether the kidneys are being affected

What to expect at your appointment

Getting a diagnosis can take time. You may need several tests and more than one specialist visit. Remember that EGPA is rare, so doctors often need to rule out other conditions first. Bring a list of your symptoms and any medicines you take. The process feels slow sometimes, but a thorough diagnosis is very important for choosing the right treatment.

Treatment

Although EGPA is serious, it can be treated effectively. Treatment focuses on calming the inflammation, preventing organ damage, and helping the immune system stop attacking the body. Most people will need treatment for a long time, but many reach remission — meaning symptoms disappear and the disease becomes quiet.

Self-care at home

• Take your medicines exactly as prescribed, even when you start feeling better.
• Keep a simple diary of your symptoms, energy levels, and any side effects.
• Get plenty of rest and pace yourself on high-energy days and low-energy days.
• Talk to your doctor before using over-the-counter medicines, including pain relievers or cold remedies.
• Follow a plan with your healthcare team for what to do if symptoms flare up again.

Medical treatments

Treatment usually starts with high-dose anti-inflammatory medicines that are often called steroids. This kind of medicine works quickly to bring down swelling and control the immune response. Once the disease is under control, the dose is gradually lowered to the smallest amount that works. For more severe cases, or when steroids alone are not enough, medications that calm or suppress the immune system may be added. These are called immunosuppressive therapies. The exact choice depends on your symptoms, which organs are involved, and your overall health. You will work closely with your doctor to find the safest and most effective plan for you.

When is surgery considered?

Surgery is not a main treatment for EGPA itself. However, some people may need sinus surgery to remove nasal polyps or open blocked sinus passages. This can improve breathing and reduce some symptoms, but it does not cure the underlying disease.

Living with this condition

Living with EGPA means learning to work with your body. Some days you may feel better, and some days you may feel more tired. Keep a regular routine, take your medicines as prescribed, and attend all follow-up appointments. Over time, you will learn to notice early signs that the disease may be flaring up, such as new shortness of breath, a rash, or tingling. Acting quickly can help prevent complications.

Lifestyle tips

• Build a small support team — healthcare providers, family, and close friends whom you can rely on.
• Find healthy ways to manage stress, like mindful breathing, gentle yoga, journaling, or talking with a counselor.
• If you have numbness in your hands or feet, protect yourself from extreme heat or cold and check for injuries, because you may not feel them.
• Avoid smoking — it can make lung symptoms worse. Ask your doctor for support if you need help quitting.
• Talk to your doctor before getting any vaccination, especially if you are taking immune-suppressing medicines.

Diet and exercise

Eat a balanced diet with plenty of fruits, vegetables, whole grains, and lean proteins. Keeping a healthy weight helps your heart and lungs. Gentle exercises like walking, stretching, or swimming can improve your strength and mood without putting too much strain on your body. If you feel tired or breathless, take it slow and let your doctor or physiotherapist guide you.

Mental health and emotional wellbeing

Living with any chronic illness can feel emotionally heavy. You may feel anxious, down, or frustrated — that is completely normal. Do not hesitate to tell your healthcare team about your feelings. They can point you toward counselors or support groups. If you ever have thoughts of harming yourself, please reach out to a crisis line or your local emergency services immediately. You deserve care and support.

Prevention

EGPA cannot currently be prevented. Because doctors do not know the exact cause, there is no proven way to stop it from happening. However, early diagnosis and good treatment can prevent many of the serious complications, and keeping regular follow-up appointments is the best way to protect your health.

Vaccines

Vaccines can help protect you from infections, which is especially important for people with chronic conditions. But certain live vaccines may not be safe while you are taking immune-suppressing treatment. Always check with your doctor or pharmacist before having any vaccination. They will advise you based on your personal situation.

Screening programmes

There is no routine screening test for EGPA in the general population. If you have unexplained asthma, sinus disease, and nerve symptoms, a doctor can evaluate you and decide if tests are needed.

Complications

If left untreated

• Nerve damage that can lead to numbness, weakness, or loss of function in the hands or feet
• Lung damage, including asthma attacks or bleeding in the lungs
• Kidney inflammation or damage, which could affect how the kidneys filter waste
• Heart problems, including inflammation of the heart muscle or its lining
• Skin ulcers from inflamed blood vessels
• Stroke or other serious blood-clotting issues in rare cases

Long-term outlook

The outlook for people with EGPA is much better than it used to be. Treatments are very effective at controlling the disease, and most people achieve remission and can live a good quality of life. Like any long-term condition, there can be relapses, but they can often be managed with prompt treatment. Staying connected to your healthcare team and following your care plan gives you the best chance of staying well.

Find support

External links open third-party websites. Ruqelo Health is not responsible for external content. Listing an organisation does not imply endorsement.