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Eosinophilic Granulomatosis with Polyangiitis or EGPA

(previously known as Churg Strauss syndrome)

Eosinophilic granulomatosis with polyangiitis, or EGPA is a form of vasculitis affecting blood vessels that used to be known as Churg Strauss syndrome or allergic granulomatous angiitis.

EGPA symptoms include fever, muscle and joint aches, tiredness, appetite loss and losing weight.

What are the symptoms of EGPA?

Because many different organ systems may be involved, many different symptoms are possible. People who have EGPA may feel: generally ill, have fevers, shortness of breath, cough, wheezing, increased nasal discharge, facial pain from sinusitis, shortness of breath from lung or heart disease, rashes, abdominal pain, blood in the stools, or muscle and joint pain ( arthritis).

EGPA in the nerves may cause an abnormal sensation, followed by loss of sensation or loss of strength. Any combination of these symptoms may be present.

Who gets EGPA?

EGPA can affect people of all ages, from children to the elderly. The average age of people with EGPA at diagnosis is about 35 to 45 years old.

What causes EGPA?

Although allergies have been thought to play a role in the development of EGPA in some people, the exact cause is unknown.

How is EGPA diagnosed?

The diagnosis of EGPA is based on a combination of factors, including:

  • Complete medical history and physical examination to exclude other conditions that may have similar symptoms
  • Presence of typical EGPA symptoms, especially the presence of asthma, the most constant feature of EGPA.
  • Blood tests that indicate abnormal blood counts with an increase in eosinophils
  • X-rays that show tissue damage or inflammation in areas such as the lungs or sinuses
  • Urinalysis, which may indicate excessive protein or blood from an abnormally high number of red blood cells ( Kidney disease caused by EGPA often does not produce symptoms; therefore, blood and urine tests are necessary to detect the problem.)

Once the diagnosis of EGPA is suspected a biopsy (tissue sample) of the affected area can confirm that inflammation of blood vessels is present. This is most easily done if there is a suspicious rash.

If easily accessible areas, such as the skin, are not affected, the diagnosis may be supported by abnormal findings of a kidney or lung biopsy. These organs would be recommended for biopsy only if there were abnormal findings during an examination or on X-rays. If the results of blood or urine tests imply kidney involvement, a biopsy to show how the kidney was damaged may be useful.

How is EGPA treated?

EGPA is generally a progressive, serious and sometimes life-threatening disorder. It requires treatment with drugs that suppress the abnormal cells of the immune system. This is intended to minimise or prevent damage to normal tissues.

Corticosteroids are the most common drugs used to treat EGPA. The most frequently used drug in this category is prednisolone. People whose nervous system, heart, kidneys or intestinal tract are not affected do extremely well with this treatment.

Other immune system suppressing drugs that may be recommended include azathioprine, cyclophosphamide and methotrexate.

WebMD Medical Reference

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