Table of ContentsView AllTable of ContentsWhat It IsSymptomsCausesDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
What It Is
Symptoms
Causes
Diagnosis
Treatment
Churg-Strauss syndrome, also calledeosinophilic granulomatosis with polyangiitis(EGPA), is a rare autoimmune condition that causes inflammation of blood vessels (vasculitis). It primarily affects the lungs but can affect other organ systems, in some cases proving fatal.
Symptoms emerge during the three stages of Churg-Strauss syndrome and depend on which organs are affected, though most people haveasthmaor asthma-like symptoms. A key feature of Churg-Strauss syndrome is a high number of white blood cells called eosinophils (hypereosinophilia).
This article explains the cause of Churg-Strauss syndrome, its symptoms, and how it is diagnosed and treated.
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What Is Churg-Strauss Syndrome?
Usually, the body releases eosinophils during allergic reactions andparasitic infections. With Churg-Strauss syndrome, large numbers of the cells are released and accumulate in the body’s tissues.
The symptoms of the disorder will correspond to the organ systems where theeosinophilsare most concentrated. For example, high numbers of eosinophils in the lungs will producerespiratory symptomslike wheezing, while a high number of the cells in the intestines will cause gastrointestinal symptoms such asabdominal pain.
What Causes Allergies? Common Allergens and Other Factors
What Are the Stages and Symptoms of Churg-Strauss ?
Symptoms of Churg-Strauss are grouped into three distinct phases, but people may not experience the phases in order and don’t always experience all three phases. In fact, recognizing the phases of Churg-Strauss symptoms is important: If the condition is diagnosed when it’s in an earlier phase, treatment can help prevent later phases from developing.
The three phases of Churg-Strauss syndrome include:
Symptoms of Churg-Strauss can vary widely, as can when they begin and how long they last. The first symptoms (which occur during the prodromal phase) are usually respiratory. These initial symptoms can include:
In the second phase, symptoms can be more generalized and include:
During the third phase, the inflammatory symptoms of vasculitis appear and can include:
More serious symptoms can develop if particular organs, such as theheartandkidneys, are involved. Many people with Churg-Strauss syndrome will experience neurological symptoms, includingpolyneuropathy.
How rare is Churg-Strauss syndrome?Churg-Strauss syndrome is very rare. The estimated average is 2.4 cases per million people each year.It occurs equally in males and females, with perhaps a slightly higher rate in males. Most cases are diagnosed between the ages of 30 and 50.
How rare is Churg-Strauss syndrome?
Churg-Strauss syndrome is very rare. The estimated average is 2.4 cases per million people each year.It occurs equally in males and females, with perhaps a slightly higher rate in males. Most cases are diagnosed between the ages of 30 and 50.
Causes of Churg-Strauss Syndrome
The exact cause of Churg-Strauss is unknown. Like otherautoimmune disorders, researchers believe that several factors, such as genetics and environment, play a role in “triggering” the immune system. Some findings include:
Causes and Risk Factors of Autoimmune Disease
How Is Churg-Strauss Syndrome Diagnosed?
Churg-Strauss is diagnosed based on a thorough history of symptoms, X-rays and computed tomography (CT) scans, and blood tests to check the levels of eosinophils. Sometimes tissuebiopsiesare taken to look for high levels of eosinophils in specific organs.
The American College of Rheumatology set the following diagnostic criteria for Churg-Strauss to help clinicians differentiate it from other types of vasculitis:
What Is the Treatment for Churg-Strauss Syndrome?
Churg-Strauss syndrome can be fatal if left untreated. Complications from chronic vasculitis can increase a person’s risk of developing serious conditions such as aneurysms, heart disease, and stroke. Diagnosing the condition early and beginning treatment can help reduce inflammation and prevent the progression to more serious symptoms.
Treatment for Churg-Strauss depends on the phase the condition is in at the time of diagnosis, which organ systems are affected, and the individual symptoms. Most people will begin treatment with medications that suppress their immune system, calledcorticosteroids. One of the most commonly prescribed corticosteroid or steroid medications isprednisone.
People with advanced disease may also need to take cytotoxic medications such as cyclophosphamide or methotrexate.
Summary
Churg-Strauss syndrome is a rare disorder, affecting about two out of a million people each year. However, it is a serious vascular disorder that can affect the lungs as well as the heart, kidneys, and nervous system.
Left untreated, Churg-Strauss can cause potentially fatal complications. Talk to your healthcare provider if you have concerns about the disorder or experience specific symptoms.
4 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Allergy Asthma & Immunology.Churg-Strauss Syndrome.National Organization for Rare Disorders.Churg-Strauss Syndrome.Paul S, Yakkali S, Teresa Selvin S, Thomas S, Bikeyeva V, Abdullah A,et al.Is Leukotriene Receptor Antagonist the Direct Cause of Churg-Strauss Syndrome in Asthmatic Patients?Cureus. 2022 Aug 14;14(8):e28018. doi: 10.7759/cureus.28018.American College of Rheumatology.1990 Criteria for the Classification of Churg-Strauss Syndrome.Additional ReadingEosinophilic Granulomatosis with Polyangiitis (EGPA/Churg-Strauss Syndrome). Vasculitis Foundation.
4 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Allergy Asthma & Immunology.Churg-Strauss Syndrome.National Organization for Rare Disorders.Churg-Strauss Syndrome.Paul S, Yakkali S, Teresa Selvin S, Thomas S, Bikeyeva V, Abdullah A,et al.Is Leukotriene Receptor Antagonist the Direct Cause of Churg-Strauss Syndrome in Asthmatic Patients?Cureus. 2022 Aug 14;14(8):e28018. doi: 10.7759/cureus.28018.American College of Rheumatology.1990 Criteria for the Classification of Churg-Strauss Syndrome.Additional ReadingEosinophilic Granulomatosis with Polyangiitis (EGPA/Churg-Strauss Syndrome). Vasculitis Foundation.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
American Academy of Allergy Asthma & Immunology.Churg-Strauss Syndrome.National Organization for Rare Disorders.Churg-Strauss Syndrome.Paul S, Yakkali S, Teresa Selvin S, Thomas S, Bikeyeva V, Abdullah A,et al.Is Leukotriene Receptor Antagonist the Direct Cause of Churg-Strauss Syndrome in Asthmatic Patients?Cureus. 2022 Aug 14;14(8):e28018. doi: 10.7759/cureus.28018.American College of Rheumatology.1990 Criteria for the Classification of Churg-Strauss Syndrome.
American Academy of Allergy Asthma & Immunology.Churg-Strauss Syndrome.
National Organization for Rare Disorders.Churg-Strauss Syndrome.
Paul S, Yakkali S, Teresa Selvin S, Thomas S, Bikeyeva V, Abdullah A,et al.Is Leukotriene Receptor Antagonist the Direct Cause of Churg-Strauss Syndrome in Asthmatic Patients?Cureus. 2022 Aug 14;14(8):e28018. doi: 10.7759/cureus.28018.
American College of Rheumatology.1990 Criteria for the Classification of Churg-Strauss Syndrome.
Eosinophilic Granulomatosis with Polyangiitis (EGPA/Churg-Strauss Syndrome). Vasculitis Foundation.
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