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Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a very rare disease that causes inflammation of the small blood vessels (vasculitis). It can affect almost every organ in the body. EGPA gets the first part of its name from one of the features of the disease: people with EGPA have an increased number of a type of white blood cell called an eosinophil.

EGPA belongs to a family of arthritic diseases called vasculitis. The word vasculitis means inflammation of blood vessels.

In EGPA, the small blood vessels that supply tissues in the lungs, sinuses, skin, nerves and almost every other organ can become inflamed. One thing that makes EGPA different from other types of vasculitis is that everyone who has EGPA also has asthma. In fact at the start of EGPA most patients notice a worsening of their asthma.

The cause of EGPA isn’t known, but it is thought to be an autoimmune disease. This means that it is thought that the disease is caused by the body’s immune system attacking its own small blood vessels and causing inflammation. It’s possible that an allergic trigger is involved in some people that misfires their immune system.

EGPA can affect anyone, but is more common in younger and middle-aged people. It affects men and women equally. It tends to occur in people who are susceptible to allergies, allergic rhinitis (hay fever) or asthma.