Eosinophilic Granulomatosis Polyangiitis
What is it?
Eosinophilic granulomatosis with polyangiitis is a disease caused by inflammation that occurs in certain types of cells in the blood or in the tissues. Everyone who gets EGPA has a history of asthma and/or allergies. It can affect many organs.
Almost all people with EGPA have increased numbers of "allergic type" blood cells called eosinophils. In EGPA, eosinophils usually make up more than 10% of the total white blood cell count. In addition, most biopsies contain clusters of cells called "granulomas" that may or may not involve blood vessels.
3 Alikes with Eosinophilic Granulomatosis Polyangiitis
Learn from others
who are experiencing
Eosinophilic Granulomatosis Polyangiitis.
Additional names
This group contains additional names:
- EGPA
- Churg Strauss
Signs & symptoms
Because EGPA can affect several different organs, there is a wide range of symptoms.
People who have EGPA may feel generally ill and fatigued or have fevers. They may lose their appetite and lose weight. Other symptoms depend on the organs or diseases involved. For example, a patient may have:
* Shortness of breath from asthma or inflammation in the air sacs and blood vessels of the lungs.
* Chest pain from disease that affects the lungs or heart.
* Rashes on the skin.
* Muscle and/or joint pain.
* Increased nasal discharge (runny nose) or facial pain from sinusitis.
* Abdominal pain or blood in the stools from intestinal tract involvement.
* Abnormal feelings, and numbness or loss of strength and feeling from nerve involvement.
Kidney disease caused by EGPA often does not have any symptoms. a person may not know about inflammation of the kidney until the kidneys begin to stop working. Therefore, if a patient have any form of vasculitis, he must have regular urinalyses (examinations of the urine).
Diagnosis
The doctor may use the following tests to diagnose EGPA:
* Medical history: to look for EGPA, especially asthma, allergies and other features of the disease.
* Physical examination: to discover which organs are involved and to rule out other illnesses that may look similar.
* Blood tests: to look for abnormal blood counts and an increase in eosinophils and special antibody testing called ANCA.
* Urinalysis: to detect whether there is too much protein, or red blood cells, in the urine.
* Imaging tests such as x-rays and computed tomography (CT) to look for any abnormalities in areas such as the lungs or sinuses.
Once the diagnosis of EGPA is suspected, a biopsy (tissue sample) is often performed to try to find eosinophils, eosinophilic granulomas and/or vasculitis. Biopsies are not required in all cases, and are only recommended when abnormal findings are seen in the exam, laboratory tests, or imaging tests.
Treatment
A variety of immunosuppressive drugs are used to treat EGPA. The doctor will choose the drug based on how the EGPA is affecting you - whether it is mild (for instance, affecting the skin or joint), or potentially life-threatening.
Glucocorticoids such as prednisone or prednisolone are the most commonly used treatments. People who have a mild disease might do extremely well on corticosteroid therapy alone. Once a patient have a dramatic improvement on this drug, the dosage is reduced to the lowest amount that keeps the disease under control.
People with EGPA that affects critical organs are usually treated with corticosteroids combined with another immunosuppressive drug, such as cyclophosphamide, methotrexate, Mycophenolate Mofetil or azathioprine.
Biologic medications are also used in EGPA such as Rituximab and Mepolizumab.
The goal of treatment is to stop all damage that comes from EGPA. This is known as "remission." Once the disease begins to improve, the doctor may slowly reduce the corticosteroid dose, and eventually stop it completely. Stopping it completely may be difficult if a person has significant asthma, as the airways can be very sensitive to corticosteroids.
The duration of maintenance immunosuppressive drugs depends on the person. In most instances, it is given for least one to two years before the healthcare provider considers slowly reducing the dosage and eventually stopping it.
☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.
Learn more about our editorial process for content accuracy.
Source of Information - We analyze big data of more than 23 million patients de-identified medical records from the following sources:
.png)
Find people who are
experiencing a similar
medical reality
Free
Alike Wisdom
Instantly get answers to medical questions with our AI, built from the collective wisdom of our community facing similar experiences
You might also like