What is EGPA?

What is Eosinophilic Granulomatosis with Polyangiitis?

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 also can be damaged.

EGPA Risk Factors

Risk factors for EGPA can include age and genetic predisposition. EGPA typically affects people between 30 and 50 years of age.

Additional risk factors include:

• Allergic rhinitis
• Asthma
• Drug sensitivities
• Environmental exposures to allergens (such as silica dust)
• Eosinophilia
• Nasal polyps
• Vasculitis

Signs & Symptoms

Symptoms of EGPA often consist of asthma and chronic sinusitis. A rash, especially on the elbows, hands and legs, also may occur. Depending on the organ affected, symptoms may include:

• Abdominal pain
• Asthma
• Blood in stool
• Chest pain
• Chronic sinusitis
• Diarrhea
• Fever
• Fatigue
• Irregular heartbeats
• Heart palpitations
• Hypertension (high blood pressure)
• Joint pain
• Muscle pain
• Nasal polyps
• Neuropathy (nerve damage often resulting in numbness, weakness, or pain of the hands and feet)

Diagnosis

A complete medical and exposure history is essential for diagnosis. Your doctor will conduct a complete physical exam and ask you questions about your lifestyle, including:

• Family history
• Job history
• Habits
• Hobbies
• Current medications
• Symptoms

When EGPA is suspected, your doctor may have you do a number of tests. To diagnose EGPA, asthma and chronic sinusitis often have to be evaluated.

Breathing and Exercise Tests

Complete pulmonary function tests: Pulmonary function tests provide a more in-depth measure how well you are breathing, as they combine spirometry with measurements of lung volumes and ability of the lung to transfer inhaled gases from the air into the bloodstream. The results of pulmonary function testing can help your doctor determine if you have EGPA. 

Imaging Tests

CT scan of the chest: A CT or CAT scan is a shortened name for computerized tomography. During a CT scan of the chest, detailed pictures are taken of cross sections or slices of the thoracic structures in your body. Thoracic structures include your lungs, heart and the bones around these areas. Sometimes intravenous contrast is administered to better see the blood vessels in the lung.

Sinus CT: When a CT scan is directed at the sinuses, it’s possible for doctors to discover abnormalities in the tissue. 

X-ray: A chest X-ray is a picture of the inside of the chest, used to evaluate the lungs, heart, and other structures of the chest. 

Lab Tests

Blood tests: Blood tests for eosinophils (a type of white blood cell) and specific autoimmune antibodies (e.g., ANA, ANCA) are also important for EGPA testing.

Urinalysis: By testing your urine, doctors are able to look for elevated levels of red blood cells or protein, which can be a sign of EGPA.

Diagnostic Procedures  

Biopsy: During a biopsy, doctors will examine a sample of tissue for signs of EGPA inflammation and vasculitis. The tissue sample will be taken from an affected area for testing.

Electrocardiogram: An electrocardiogram (EKG or ECG) is a non-invasive test that reads electrical signals from your heart as it contracts and relaxes. By monitoring your heart’s rhythm, doctors are able to look for signs of abnormalities or damage, which could be indications of EGPA. 

Treatment

Treatment of eosinophilic granulomatosis with polyangiitis (EGPA) depends on the severity of the disease.

Medications

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 or benralizumab, which can help to reduce the number of eosinophils in the body.

Other immunomodulator medications such as azathioprine or cyclophosphamide are also given. These help reduce the body's autoimmune response.

Clinical Trials

Clinical trials help determine new treatment options for diseases and conditions. Patients with EGPA have access to clinical trials and should speak with their physician to determine what trials might work best for them.