Learn About Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Key Facts

• EGPA is an ultra-rare disease, affecting an estimated 50,000 individuals in the United States.
• It is a form of vasculitis, which means inflammation of the blood vessels that can reduce blood flow causing tissue and organ damage.
• Previously called Churg-Strauss syndrome, it was renamed EGPA in 2010.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare form of vasculitis, characterized by inflammation within small blood vessels. This inflammation results in blood flow restriction, which can cause organ damage throughout the body if left untreated. EGPA is a serious, but treatable disease.  Relapses can occur so it is important to maintain ongoing communication with your healthcare provider about management of this chronic disease.

What Causes EGPA?

It is not known what causes EGPA. Typically, the immune system defends the body but in auto-immune diseases, such as vasculitis, the immune system increases activity and attacks healthy tissues for reasons we don’t quite understand. 

How EGPA Affects Your Body

In most patients, EGPA affects your lungs, but it can also affect other organs in the body. EGPA affects people differently, depending on which organs are affected by the disease as well as the severity.  It is referred to by the following phases:

• Phase One. Typically presents as adult-onset asthma. These asthmatic symptoms usually begin years before any other symptoms of EGPA appear.  Other early warning symptoms include nasal polyps and allergic rhinitis.  
• Second phase. Observed by excessive numbers of eosinophils in the blood or tissue. Eosinophils are a type of white blood cell. These cells usually account for less than 5% of the total white blood cell count, in EGPA they can be as high as 60% of the total white blood cell count. 
• Phase three. Involves vasculitis (inflammation of a blood vessel or blood vessels) and may include damage to the lungs, skin, nerves, kidneys and other organs.

Who Is at Risk for EGPA Lung Disease?

Anyone can get EGPA.  It equally affects men and women and can occur at any age, though the average age someone is diagnosed is between 35 and 50 years old.