What is Eosinophilic Granulomatosis with Polyangitis?
Eosinophilic granulomatosis with polyangiitis (EGPA)is a rare disease caused by blood vessel inflammation, which disrupts blood supply and damages vital organs and tissues.
What are the Causes of Eosinophilic Granulomatosis with Polyangitis?
The exact cause of eosinophilic granulomatosis with polyangiitis is unknown. It is most likely due to an overactive immune system triggered by genes and environmental factors, such as allergens or certain drugs. The immune system defends the body against foreign bodies (germs, bacteria) that can cause infections, illness, and other injuries to the body.
What are the Symptoms of Eosinophilic Granulomatosis with Polyangitis?
Some of the symptoms of eosinophilic granulomatosis with polyangitis include:
- Fever
- Rashes on the skin
- Fatigue
- Sudden rapid weight loss
- Muscle and joint pain
- Chest pain
- Increasing shortness of breath or coughing
- Abdominal pain
- Blood in the stools
- Sudden loss of strength in the hands or feet
Diagnosis of Eosinophilic Granulomatosis with Polyangitis
Your doctor will review your medical history and symptoms and based on this a physical examination will be performed. Your doctor may also recommend the following diagnostic tests:
- Urinalysis: To detect excessive protein or the presence of red blood cells in the urine
- Blood Tests: To check for abnormal blood counts such as an increase in eosinophils and special antibody testing called ANCA (Antineutrophil cytoplasmic antibody)
- Tissue Biopsy: To examine a sample from the affected vessel or organ under a microscope for signs of inflammation or tissue damage
- Imaging studies (X-rays and computed tomography): To check for any abnormalities in areas like the lungs or sinuses
Treatment for Eosinophilic Granulomatosis with Polyangitis
EGPA is treated with a range of immunosuppressive medicines (drugs that inhibit the immune system). The prescription will be chosen by your doctor based on the severity of your EGPA symptoms, whether they are mild or potentially life-threatening.
The most prevalent therapies involve the use of glucocorticoids like prednisone or prednisolone. Patients with mild illness (those whose condition does not impact the neurological system, heart, kidneys, or intestinal tract, or show other symptoms of severe disease) may benefit greatly from corticosteroid medication alone. Patients with EGPA affecting critical organs may be treated with corticosteroid in combination with another immunosuppressive treatment. Biologic medications are also used in EGPA such as Rituximab and Mepolizumab. The goal of treatment is to halt the damage caused by EGPA.
