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Diseases > Eosinophilic Granulomatosis with Polyangiitis (EGPA) > What is it?

What is EGPA?

Eosinophilic granulomatosis with polyangiitis (EGPA) is also known as Churg-Strauss syndrome. It is a rare disease that causes inflammation of the small blood vessels. EGPA gets its name from one of the features of the disease: people with EGPA have an increased number of white blood cells known as “eosinophils.”

EGPA belongs to a family of diseases called “vasculitis”. All diseases in this family involve inflammation of blood vessels. In EGPA, this vasculitis results in inflammation of the small blood vessels that supply tissues in the lungs, sinuses, skin, nerves and other organs.  It can affect almost every organ in the body. One thing that makes EGPA different from other types of vasculitis is that everyone who has EGPA also has asthma.

What causes EGPA?

We do not know the cause of EGPA, but it is thought to be an autoimmune disease. This means that something causes your immune system to go awry. It begins to attack small blood vessels and causes inflammation. An allergic trigger may cause the number eosinophils in the blood to rise, but we don’t know why this happens.

The word inflammation comes from the Latin word inflammare which means to light on fire. You can think of it like this: in people with EGPA, small blood vessels in certain areas of the body are “on fire” and are eventually damaged. This can cut off blood supply to vital organs.

Who gets EGPA?

EGPA is very rare. It can affect anyone, but is more common in younger and middle-aged people (40-50 years old). It affects men and women equally. It also tends to occur in people who are susceptible to allergies, allergic rhinitis or asthma.

Chronic cocaine users often have symptoms similar to EGPA. If you use narcotic drugs such as cocaine, tell your doctor. It is important for them to know this. Remember, everything you tell your doctor is confidential.

How is EGPA diagnosed?

There are two key features that help make the diagnosis of EGPA: a history of asthma and a high number of eosinophils in the blood.

EGPA can sometimes be challenging to diagnose because the symptoms may come and go. A rheumatologist – a specialist in arthritis and autoimmune diseases – is in the best position to diagnose EGPA. Your rheumatologist will take a complete history and perform a thorough physical examination. This is usually followed by blood tests, x-rays and other tests.

What tests are done to diagnose EGPA?

A variety of blood tests help confirm the diagnosis of EGPA. If the skin or lungs are involved, a tissue biopsy may be done. This involves taking a very small sample of tissue through a needle.

These are some of the other tests that can help diagnose EGPA:

  • Complete blood count (CBC) including eosinophils
  • Anti-neutrophil cytoplasmic antibody (ANCA) test
  • Erythrocyte sedimentation rate (ESR)
  • IgE test
  • C-reactive protein (CRP)
  • Creatinine and urinalysis to look for kidney involvement
  • Chest x-ray to look for lung involvement
  • EMG or Nerve Conduction Studies can be performed to look for nerve involvement
  • Echocardiogram to look for heart involvement

Read more – What is it going to do to me?