EGPA (formerly Churg-Strauss syndrome)

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.

The first sign of EGPA typically is 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 for EGPA, 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.

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:

• Allergic stage. This first stage often lasts for years. It typically includes new or worsening asthma, nasal allergies and repeated sinus infections.
• 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. Eosinophils can build up in tissues and organs, causing damage. Common issues in this stage include lung symptoms such as coughing or wheezing and digestive issues such as stomach pain or bleeding, nausea and vomiting.
• Vasculitic stage. This final stage involves vasculitis, which is 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.

These stages may overlap, and not everyone goes through all three stages.

Other symptoms may include:

• 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.

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.

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.

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.

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. Complications may include:

• Nerve damage, also called neuropathy. EGPA can damage the nerves in your hands and feet. This can cause numbness, burning, pain and muscle weakness.
• Heart disease. Inflammation caused by EGPA can affect the heart and lead to chest pain, heart attack or heart failure.
• Kidney damage. If EGPA affects your kidneys, it can cause a condition called glomerulonephritis. This can lessen your kidneys' ability to filter waste, leading to serious health issues.

There is no known way to prevent EGPA since the exact cause is unknown. But you can take these steps to reduce the risk of complications:

• Get regular medical care if you have asthma or allergies.
• Tell your healthcare professional about any new or worsening symptoms.
• Follow your treatment plan closely if you are diagnosed with EGPA.
• Avoid known triggers that cause allergy or asthma flare-ups.

Early diagnosis and treatment are the best ways to prevent serious health issues from this condition.