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Eosinophilic Granulomatosis with Polyangiitis

(Churg-Strauss Syndrome; Allergic Granulomatosis and Angiitis)


Alexandra Villa-Forte

, MD, MPH, Cleveland Clinic

Reviewed/Revised May 2022 | Modified Sep 2022
Topic Resources

Eosinophilic granulomatosis with polyangiitis is inflammation of small- and medium-sized blood vessels that damages organs and that usually occurs in adults with a history of asthma, nasal allergies, nasal polyposis, or a combination.

  • 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.

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.


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.


  • 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 Antibodies (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 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.


The prognosis is poor for people with eosinophilic granulomatosis with polyangiitis whose heart, brain, spinal cord, or nerves are affected.


  • 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
Deltasone, Predone, RAYOS, Sterapred, Sterapred DS
Azasan, Imuran
RIABNI, Rituxan, RUXIENCE, truxima
Jylamvo, Otrexup, Rasuvo, RediTrex, Rheumatrex, Trexall, Xatmep
Cyclophosphamide, Cytoxan, Neosar
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