Eosinophilic Granulomatosis with Polyangiitis: A Guide

A rare autoimmune condition causing inflammation in small and medium-sized blood vessels, often accompanied by asthma.
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Autoimmune Disorders | Vasculitis | Asthma | EGPA | Autoimmune | Steroids
Prepared by Lee Cheng, reviewed by Dr. Helena Rodriguez

Eosinophilic Granulomatosis With Polyangiitis FAQ


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What is eosinophilic granulomatosis with polyangiitis (EGPA)?

ACR Open Rheumatol. 2021 Feb; 3 (2): 101–110. Eosinophilic granulomatosis with polyangiitis (EGPA) is part of a group of vasculitides commonly referred to as antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), in addition to granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and renal‐limited vasculitis.

What is granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (GPA) is a rare condition where the blood vessels become inflamed. It mainly affects the ears, nose, sinuses, kidneys and lungs. Anyone can get it, including children, but it's most common in adults and older people.

What are the symptoms of eosinophilic granulomatosis with polyangiitis?

In people with eosinophilic granulomatosis with polyangiitis, asthma, nasal allergies, nasal polyposis, or a combination may develop or worsen over many years. People may sneeze and have a persistently runny nose and itchy eyes. Inflammation of the sinuses may cause facial pain. Later, people may feel generally ill and tired.

How do you test for eosinophilic granulomatosis with polyangiitis?

There is no specific blood test for eosinophilic granulomatosis with polyangiitis. Investigations usually include: Imaging studies may include X-rays of the lungs and sinuses, and electrocardiogram ( ECG ). Skin biopsy and kidney biopsy may demonstrate the diagnostic combination of tissue eosinophilia, vasculitis and granulomas.

What is eosinophilic granulomatosis with polyangiitis?

Eosinophilic granulomatosis with polyangiitis ( EGPA ), formerly known as allergic granulomatosis, is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels ( vasculitis) in persons with a history of airway allergic hypersensitivity ( atopy ). It usually manifests in three stages.

How do you treat eosinophilic granulomatosis with polyangiitis?

Corticosteroids (such as prednisone) are given to treat eosinophilic granulomatosis with polyangiitis. These drugs can reduce inflammation. Another drug that suppresses the immune system (immunosuppressant) is also used. Azathioprine, rituximab, or methotrexate may be used. Cyclophosphamide is used when the symptoms are severe.

Can eosinophilic granulomatosis cause an allergic reaction?

An increased number of eosinophils is called eosinophilia, and the increase suggests that an allergic reaction may be part of the disorder. In people with eosinophilic granulomatosis with polyangiitis, asthma, nasal allergies, nasal polyposis, or a combination may develop or worsen over many years.

Eosinophilic Granulomatosis With Polyangiitis References

If you want to know more about Eosinophilic Granulomatosis With Polyangiitis, consider exploring links below:

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