Living with eosinophilic granulomatosis with polyangiitis

Overview

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare condition where the immune system mistakenly attacks small blood vessels. This causes inflammation that can affect the lungs, skin, nerves, and other organs. It is sometimes called Churg-Strauss syndrome. With proper treatment, many people with EGPA manage their symptoms and lead active lives.

No, EGPA is very rare. It is estimated to affect about 1 to 3 people per million each year.

EGPA can occur at any age, but it most often starts between ages 40 and 60. It is slightly more common in people with asthma, and some studies show it may be more common in women.

Symptoms

Common symptoms

• Wheezing, shortness of breath, or a cough that does not go away
• Pain or pressure in your sinuses, nasal congestion, or repeated sinus infections
• A rash, such as small red or purple spots, especially on the legs
• Numbness, tingling, or weakness in your hands or feet
• Joint pain or muscle aches
• Feeling very tired (fatigue), fever, or weight loss without trying
• Belly pain, nausea, or diarrhea

Symptoms in children

• EGPA is extremely rare in children. When it does happen, symptoms may be similar to adults, including asthma, rash, and nerve symptoms. Children and teenagers may also have trouble growing or gaining weight. Any child with these symptoms should see a pediatric specialist.

Symptoms in older adults

• Older adults may have more severe symptoms at the time of diagnosis. They may also have other health conditions such as high blood pressure or diabetes, which can make management more complex. Treatment decisions are tailored to the individual.

Causes

Main causes

• The exact cause of EGPA is unknown, but it is an autoimmune disease, meaning the body's immune system mistakenly attacks its own blood vessels.
• A combination of genetic factors and something in the environment may trigger the disease. Infections, allergies, or certain medicines might play a role, but this is not yet fully understood.

Risk factors

• Having asthma – this is the most common risk factor, and it usually develops years before EGPA appears.
• Chronic sinusitis or allergic rhinitis (hay fever)
• Being an adult between 40 and 60 years old

When to see a doctor

See a doctor urgently if:

• If you have any symptoms that could be life-threatening (listed under emergency above), call your local emergency number right away.
• If you have blood in your urine or bowel movements, or are coughing up blood, seek urgent care.
• If you notice sudden loss of vision or double vision, this is an emergency.

Book a routine appointment if:

• If you have asthma that is becoming harder to control, or repeated sinus infections, make an appointment with your doctor.
• If you develop a rash, unexplained numbness, or any new symptoms that last more than a week, bring them to your doctor's attention.
• If you have been diagnosed with EGPA, keep all follow-up appointments with your specialist team.

Diagnosis

There is no single test for EGPA. Doctors diagnose it by looking at your medical history, doing a physical exam, and checking results from multiple tests. They may also rule out other conditions with similar symptoms.

Tests that may be done

• Blood tests to check for high levels of eosinophils (a type of white blood cell) and certain antibodies
• Imaging tests, such as chest X-rays or CT scans, to look at your lungs and sinuses
• Tests to check how your organs are working, such as urine tests or nerve conduction studies
• A biopsy, which is a tiny sample of tissue taken from an affected area like the skin or lung, to look for inflammation

What to expect at your appointment

You will likely see a rheumatologist (a specialist in autoimmune diseases) or another specialist who knows about rare conditions. Diagnosis can take time, and you may need several appointments and tests. Try to keep a list of your symptoms and when they started, and tell your doctor about any asthma or allergy history.

Treatment

Treatment for EGPA aims to reduce inflammation, control symptoms, and protect your organs. Most people need a combination of medicines, and regular monitoring is important. The best plan depends on how severe your disease is, which organs are affected, and your personal health.

Self-care at home

• Take your medicines exactly as prescribed, even when you feel well.
• Keep track of your symptoms and report any changes to your healthcare team.
• Avoid things that trigger your asthma, such as smoke, dust, or strong fumes.
• Get enough rest, and pace yourself during busy days.
• Keep a healthy routine – this includes eating balanced meals, staying active, and sleeping well.
• Stay up to date with vaccinations that your doctor recommends.

Medical treatments

Doctors often use steroid medicines to quickly reduce inflammation. For more severe disease, they may prescribe medicines that calm the immune system (immunosuppressants) or targeted biologic therapies. These treatments are chosen by a specialist team and are tailored to each person. You will have regular blood tests to monitor the effects of treatment. Do not stop any medication without consulting your doctor, as this can lead to a flare-up.

When is surgery considered?

Surgery is rarely needed for EGPA itself. In rare cases, it may be needed to treat complications such as a severely affected bowel or bleeding. Your specialist team will discuss any surgical options with you if they become necessary.

Living with this condition

Living with EGPA requires ongoing care. You will see your specialist regularly to check that your treatment is working. It is normal to have good days and bad days. Learn to listen to your body, and give yourself permission to rest when you need to. Keep a symptom diary – this can help you and your doctor spot patterns and adjust your plan.

Lifestyle tips

• Quit smoking, if you smoke, because smoking makes asthma and blood vessel damage worse.
• Limit alcohol, as it can interfere with your medicines and health.
• Protect your skin from cold and injury, especially if you have numbness in your fingers or toes.
• Build a support network of family, friends, and healthcare professionals.
• Consider relaxation techniques like gentle breathing exercises or meditation to manage stress.

Diet and exercise

Eating a balanced diet can support your overall health. There is no special diet that cures EGPA, but certain foods may help reduce inflammation, such as fruits, vegetables, whole grains, and oily fish. Exercise is important for staying strong and improving energy, but choose activities that suit your fitness level. For example, walking, swimming, or yoga can be good options. Talk to your doctor or a physiotherapist if you are unsure what exercise is safe for you.

Mental health and emotional wellbeing

Living with a chronic illness can be emotionally challenging. You may feel anxious, sad, or stressed, and that is completely understandable. It is important to talk about how you feel. If you are struggling, ask your healthcare team about counseling or support groups. Remember: if you ever feel hopeless or have thoughts of self-harm, please reach out for help immediately. Call your local mental health crisis line or your local emergency number.

Prevention

EGPA cannot be prevented, because it is an autoimmune disease and the exact cause is unknown. However, you can help prevent flare-ups by taking your medication as prescribed, avoiding asthma triggers, and attending regular check-ups with your specialist.

Vaccines

People with EGPA are often advised to stay up to date with vaccines, such as the annual flu vaccine and other recommended vaccines. Talk to your healthcare team about which vaccines are right for you, because some vaccines contain live viruses and may not be safe if your immune system is suppressed.

Screening programmes

There is no routine screening test for EGPA. Regular follow-up with your doctor is the best way to monitor your health and catch any early signs of problems.

Complications

If left untreated

• Lung damage, leading to permanent breathing problems
• Nerve damage that causes weakness or loss of feeling in your arms or legs
• Kidney disease or kidney failure
• Heart problems, including inflammation of the heart muscle or blood vessels
• Bowel problems that may require surgery

Long-term outlook

With early diagnosis and proper treatment, many people with EGPA improve significantly. The condition may become quiet for long periods, and some people can eventually reduce their medication under medical supervision. EGPA can be managed, but it does require ongoing care. Working closely with your specialist team gives you the best chance of staying healthy and living a fulfilling life.

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