Eosinophilic Granulomatosis With Polyangiitis (EGPA, formerly Churg-Strauss Syndrome)

What is EGPA (formerly Churg-Strauss syndrome)?

EGPA stands for “eosinophilic granulomatosis with polyangiitis.” This is a long name, but it’s more specific than the former name, Churg-Strauss syndrome. EGPA is a rare disorder that occurs in people with a history of severe allergies and/or asthma. It affects your blood vessels, especially the ones in your lungs.

Let’s break the name down:

• Eosinophilic: Eosinophilia is an unusually high number of eosinophils in your blood. Eosinophils are a type of white blood cell that supports your immune system. Your eosinophil levels rise when your immune system is highly active. With EGPA, eosinophils are often double the norm.
• Granulomatosis: Granulomas are small masses of immune cells that can form in your tissues when those tissues are inflamed. They’re another tool of your immune system, meant to help “wall off” infections and other threats. Granulomatosis is the process of granulomas forming.
• Polyangiitis: Polyangiitis is a type of vasculitis, or chronic inflammation in your blood vessels. It affects the many small-to-medium-sized blood vessels throughout your body (rather than the relatively few larger ones). Poly means many, and angiitis is another term for vasculitis.

EGPA seems to start with inflammation in your respiratory system, related to allergies or asthma. Eosinophilia and granulomatosis are signs that your immune system is in overdrive. Polyangiitis is a dysfunctional immune response. It’s inflammation that’s automatic, ongoing and has no real target.

How does eosinophilic granulomatosis with polyangiitis (EGPA) affect my body?

Chronic vasculitis weakens the walls of your blood vessels. This can cause them to stretch, leading to an aneurysm, or to break, leading to internal bleeding. Inflammation can also cause swelling and scarring that narrows your blood vessels. This can restrict or stop the blood flow to your tissues and organs.

As your smaller blood vessels travel throughout your body, polyangiitis can cause symptoms throughout your body. But it especially affects the organs that rely on many small blood vessels. EGPA affects your respiratory system the most. It may also affect your kidneys, intestines, heart or nerves.

What are the symptoms of EGPA (formerly Churg-Strauss syndrome)?

EGPA develops gradually, and symptoms tend to occur in phases over several years.

Common early symptoms (phase 1) include:

• Adult-onset asthma with coughing, wheezing and shortness of breath.
• Chronic hay fever with sneezing, nasal itching and congestion.
• Chronic sinusitis (inflammation in your sinuses) and sinus pressure.
• Nasal polyps (noncancerous growths in your nose).
• Generalized joint pain and/or muscle pain.
• Fever, malaise (general unwellness) and fatigue.

As the condition progresses, you may develop eosinophilic infiltrates — clusters or masses of eosinophils that infiltrate your lungs and other tissues. This can lead to additional (phase 2) symptoms, including:

• Chest pain and difficulty breathing.
• Heart palpitations.
• Skin rashes or lesions.
• Gastrointestinal symptoms, like stomach pain and diarrhea.

Vasculitis (polyangiitis) is usually the last stage (phase 3) of the disease. It can cause:

• Symptoms of internal bleeding, like blood in your stool or coughing up blood or discolored spots under your skin.
• Symptoms of nerve inflammation or damage, like numbness and tingling, weakness or nerve pain.
• Symptoms of heart disease, like irregular heartbeats, faintness, shortness of breath and swelling (edema).

Polyangiitis will often affect your kidneys as well, but usually without symptoms.

What causes EGPA (formerly Churg-Strauss syndrome)?

Eosinophilic polyangiitis with granulomatosis (EGPA) develops from an overreaction from your immune system. Your immune system generates the inflammation in your blood vessels, the granulomas in your tissues and the eosinophilia that cause your symptoms. But researchers aren't sure why this happens.

Diseases that involve chronic inflammation from your immune system are sometimes called autoimmune diseases. One common feature of autoimmune diseases is that your body can develop antibodies that target certain cells in your body with inflammation. This may be one factor in EGPA.

Researchers have grouped EGPA together with a handful of similar conditions that all involve a certain autoimmune antibody, called ANCA. They call these conditions ANCA-associated vasculitis, or AAV. However, only about 40% of people with EGPA have the ANCA antibody, so it’s not the only factor.

Eosinophilia seems to be a separate cause of symptoms that often occur long before vasculitis does. When eosinophils accumulate in your tissues, they trigger inflammation and damage them. Eosinophilia is related to asthma and allergies. People with asthma are 34 times more at risk of EGPA than others.

What are the serious complications of EGPA (formerly Churg-Strauss syndrome)?

If it goes untreated, EGPA can cause serious damage to your organs and tissues, including:

• Pleural effusion.
• Pericardial effusion.
• Peripheral neuropathy.
• Chronic kidney disease.
• Chronic respiratory failure.
• Congestive heart failure.

How is eosinophilic granulomatosis with polyangiitis (EGPA) diagnosed?

EGPA can be tricky to diagnose. You might have different symptoms at different times, and you and your healthcare provider might not connect them all together right away. Symptoms of EGPA can also resemble many other conditions. Your provider might suspect EGPA if you have most of these:

• A history of asthma or chronic hay fever.
• Evidence of eosinophilia on a blood test.
• Evidence of eosinophilic infiltrates on imaging tests.
• Evidence of granulomas and/or vasculitis on a tissue biopsy.

What is the treatment for EGPA (formerly Churg-Strauss syndrome)?

Treatment for EGPA begins with a high dose of corticosteroids to reduce inflammation and eosinophils. When they’ve reduced enough to relieve your symptoms, your disease is in remission. At this point, your provider will begin reducing your dose. Most people continue to take a low dose of corticosteroids to maintain remission.

Your provider may prescribe additional medications if corticosteroids alone aren’t effective enough, or if you want to avoid taking a high dose of corticosteroids.

Additional medications include immunosuppressants and biologics. Mepolizumab is the first U.S. Food and Drug Administration (FDA)-approved biologic therapy for EGPA. Benralizumbab, another biologic agent, performed equally well in a recent clinical trial. These recent breakthroughs in research offer new treatment options for people with EGPA.

Is there any way to prevent eosinophilic granulomatosis with polyangiitis (EGPA)?

As we don’t know what causes EGPA, we don’t know any way to prevent it. However, recognizing it and treating it earlier can prevent it from worsening. By paying close attention to your symptoms and reporting them to your healthcare provider, you may be able to prevent the complications of EGPA.

What is the outlook (prognosis) for EGPA (formerly Churg-Strauss syndrome)?

EGPA is treatable, but not curable. Most people can manage their symptoms with medications and even achieve remission, meaning symptoms go away. But when you stop treatment, symptoms may start again (relapse). Your healthcare provider will continue to monitor your condition throughout your life.

Can you live a long life with EGPA?

With effective treatment, you can have a normal life expectancy with EGPA. In the advanced stages of the disease, complications like organ failure can affect your life expectancy. But treatment can often stop or reverse organ failure. With treatment, EGPA survival rates after five years are over 80%.

How should I take care of myself while living with EGPA?

Medications for EGPA reduce your immunity, which makes it easier for you to get sick and harder for you to get better. When you’re taking these medications, you’ll need to take extra care to protect yourself from common illnesses, because you won’t be able to “bounce back” as easily as before.

Long-term treatment with corticosteroids can also cause side effects, including high blood sugar and weight gain, bone thinning and mood changes. Your provider will work with you to manage side effects while managing EGPA. Stay in touch with your provider about all your symptoms, especially new ones.