Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Eosinophilic granulomatosis with polyangiitis (EGPA) was previously known as Churg-Strauss Syndrome. It has also been called allergic granulomatosis and angiitis.

EGPA is an inflammatory condition, characterized by asthma, high levels of eosinophils (a type of white blood cell) and sinus disease. Neuropathy (nerve damage, often resulting in numbness, weakness or pain of the hands and feet) and vasculitis occur in at least one organ. Vasculitis refers to inflammation of the blood vessel wall. When small and medium size arteries are affected, multiple organ systems can be affected. In EGPA, the lungs and skin are most often involved. The heart, gastrointestinal tract, kidneys and nervous system can also be damaged.

EGPA Symptoms

Symptoms of eosinophilic granulomatosis with polyangiitis (EGPA) often consist of asthma and chronic sinusitis. Chronic sinusitis is inflammation of the sinuses. A rash, especially on the elbows, hands and legs, may also occur. Depending on the organ affected, symptoms may include neuropathy (nerve damage often resulting in numbness, weakness, or pain of the hands and feet), abdominal pain, diarrhea and hypertension (high blood pressure).

Diagnosing EGPA

To diagnose eosinophilic granulomatosis with polyangiitis (EGPA), asthma and chronic sinusitis often have to be evaluated. This evaluation includes lung function tests and CT scans of the chest and sinuses. Blood tests for eosinophils (a type of white blood cell) and specific autoimmune antibodies (e.g., ANA, ANCA) are also important. In addition, a sample may be taken from an involved area for further diagnosis. This is called a biopsy.

EGPA Treatment

Treatment of eosinophilic granulomatosis with polyangiitis (EGPA) depends on the severity of the disease. Treatment often includes oral steroids to reduce inflammation. When there are more widespread symptoms, if the disease fails to respond to steroids or if the person can’t be decreased to lower doses of oral steroids or develops side effects, steroid sparing agents are considered. Steroid sparing agents for EGPA may include targeted antibody therapies like mepolizumab (anti IL-5). Other immunomodulator medications such as azathioprine or cyclophosphamide are also given. These help reduce the body's autoimmune response.