The cause is unknown.
At first, people may have a runny nose or asthma for months or years or have sinus pain, followed by various symptoms, depending on which organs are affected.
Doctors base the diagnosis on symptoms and results of a physical examination, blood tests, imaging tests, and biopsy.
Corticosteroids in combination with another drug that suppresses the immune system may be used.
(See also Overview of Vasculitis Overview of Vasculitis Vasculitic disorders are caused by inflammation of the blood vessels (vasculitis). Vasculitis can be triggered by certain infections or drugs or can occur for unknown reasons. People may have... read more 
.)
Eosinophilic granulomatosis with polyangiitis can occur in people of all ages. The average age at the time of diagnosis is 48. Affected people develop asthma Asthma Asthma is a condition in which the airways narrow—usually reversibly—in response to certain stimuli. Coughing, wheezing, and shortness of breath that occur in response to specific triggers are... read more 
, nasal allergies Year-Round Allergies Year-round (perennial) allergies result from indoor exposure to airborne substances (such as house dust) that are present throughout the year. The nose is congested, itchy, and sometimes runny... read more , nasal polyposis Nasal Polyps Nasal polyps are fleshy outgrowths of the mucous membrane of the nose. Nasal polyps are more likely to develop in people who have allergies or asthma. Some of the symptoms caused by polyps are... read more 
(when numerous polyps develop in the nose), or a combination in adulthood. The cause of eosinophilic granulomatosis with polyangiitis is unknown.
Inflammation that can affect small- and medium-sized blood vessels (vasculitis) may affect any organ. The peripheral nervous system, sinuses, skin, joints, lungs, digestive tract, heart, and kidneys are most commonly affected. Collections of immune cells that cause inflammation (called granulomas) may form raised bumps (nodules) in affected tissue. Granulomas can destroy normal tissue and interfere with functioning. They may also cause lumps to form under the skin. People also have an increased number of eosinophils (a type of white blood cell) in their blood and body tissues. An increased number of eosinophils is called eosinophilia, and the increase suggests that an allergic reaction may be part of the disorder.
Symptoms
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. They may have fevers or night sweats or lose their appetite and lose weight. Other symptoms depend on which organs are affected and may include the following:
Muscle and joint pain
Shortness of breath, asthma, and sinusitis
Chest pain
Rashes
Abdominal pain and diarrhea
Blood in the stool
Abnormal sensations, numbness, or weakness in a limb, often sudden
Confusion, seizures, and coma
Any combination of these symptoms may occur. Symptoms may occur in episodes. In subsequent episodes, people may have the same symptoms as the first episode or different ones.
Inflammation of the kidneys may not cause symptoms until the kidneys malfunction and kidney failure Overview of Kidney Failure Kidney failure is the inability of the kidneys to adequately filter metabolic waste products from the blood. Kidney failure has many possible causes. Some lead to a rapid decline in kidney function... read more develops. Other complications include heart failure Heart Failure (HF) Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more 
, heart attack Acute Coronary Syndromes (Heart Attack; Myocardial Infarction; Unstable Angina) Acute coronary syndromes result from a sudden blockage in a coronary artery. This blockage causes unstable angina or a heart attack (myocardial infarction), depending on the location and amount... read more 
, pericarditis Acute Pericarditis Acute pericarditis is inflammation of the pericardium (the flexible two-layered sac that envelops the heart) that begins suddenly, is often painful, and causes fluid and blood components such... read more 
, and heart valve disorders Overview of Heart Valve Disorders Heart valves regulate the flow of blood through the heart's four chambers—two small, round upper chambers (atria) and two larger, cone-shaped lower chambers (ventricles). Each ventricle has... read more 
.
Diagnosis
A doctor's evaluation
Blood and urine tests
Imaging tests
Biopsy (of skin, muscle, and, sometimes, lung tissue)
Early diagnosis and treatment of eosinophilic granulomatosis with polyangiitis help prevent severe organ damage.
No single test can confirm the diagnosis. The diagnosis of eosinophilic granulomatosis with polyangiitis is made by recognizing the combination of typical symptoms and results of the physical examination and other tests.
Blood tests are done. Doctors determine how many eosinophils are in the blood. Eosinophils are produced during allergic reactions, and their number increases when eosinophilic granulomatosis with polyangiitis is present. Doctors also look for certain antibodies Antibodies One of the body's lines of defense ( immune system) involves white blood cells (leukocytes) that travel through the bloodstream and into tissues, searching for and attacking microorganisms and... read more 
(antineutrophil cytoplasmic antibodies) that may be present. Doctors measure how quickly red blood cells (erythrocytes) drop to the bottom of a test tube (erythrocyte sedimentation rate, or ESR). A fast rate suggests inflammation. Doctors also measure levels of C-reactive protein (which the liver produces in response to bodywide inflammation). A high C-reactive protein level also suggests inflammation. Urine tests are done to determine whether the kidneys are affected.
A chest x-ray is done to look for inflammation in the lungs. Doctors also do echocardiography of the heart Echocardiography and Other Ultrasound Procedures Ultrasonography uses high-frequency (ultrasound) waves bounced off internal structures to produce a moving image. It uses no x-rays. Ultrasonography of the heart (echocardiography) is one of... read more 
to look for signs of heart failure.
A sample of inflamed tissue is taken and examined under a microscope (biopsy). A biopsy can show whether the tissue contains eosinophils or granulomas. Sometimes a biopsy of lung tissue is necessary. It may require hospitalization.
Prognosis
The prognosis is poor for people with eosinophilic granulomatosis with polyangiitis whose heart, brain, spinal cord, or nerves are affected.
Treatment
Corticosteroids and other immunosuppressants
Corticosteroids (such as prednisone) are given to treat eosinophilic granulomatosis with polyangiitis. These drugs can reduce inflammation. Another drug that suppresses the immune system Overview of the Immune System The immune system is designed to defend the body against foreign or dangerous invaders. Such invaders include Microorganisms (commonly called germs, such as bacteria, viruses, and fungi) Parasites... read more (immunosuppressant) is also used. Azathioprine, rituximab, or methotrexate may be used. Cyclophosphamide is used when the symptoms are severe.
After symptoms resolve, the dose of the drugs is gradually reduced, and, after a while, the drugs may be stopped. If necessary, they can be started again. These drugs, especially when taken for a long time, can have serious side effects.
People with eosinophilic granulomatosis with polyangiitis should learn about their disorder. Then they can recognize any new symptoms and report them immediately to the doctor.
More Information
The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
Vasculitis Foundation: Provides information for patients about vasculitis, including how to find a doctor, learn about research studies, and join patient advocacy groups
Drugs Mentioned In This Article
| Generic Name | Select Brand Names |
|---|---|
prednisone |
Deltasone, Predone, RAYOS, Sterapred, Sterapred DS |
azathioprine |
Azasan, Imuran |
rituximab |
RIABNI, Rituxan, RUXIENCE, truxima |
methotrexate |
Otrexup, Rasuvo, RediTrex, Rheumatrex, Trexall, Xatmep |
cyclophosphamide |
Cyclophosphamide, Cytoxan, Neosar |
