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Churg-Strauss Syndrome


Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyarteritis (EGPA) is a type of vasculitis. These are disorders characterized by inflammation of blood vessels. This inflammation often affects the lungs, skin, nerves and stomach. Asthma is the most common sign of CSS/EGPA. Symptoms may also include other lung problems, allergic rhinitis (hay fever), skin rashes, stomach pain and numbness in the hands and feet.


Symptoms & Diagnosis

CSS/EGPA can result in a variety of symptoms, from mild to very severe and life-threatening. Symptoms often occur in stages, though not all people will develop these stages in the same order. If caught early, later stages can be prevented through treatment.

The earliest stage is the allergic stage. Common symptoms in this stage include:
•    Coughing, wheezing, shortness of breath
•    Itchy, runny and stuffy nose
•    Sinus pressure and pain that can be associated with nasal polyps

The next stage is hypereosinophilia, meaning that there are abnormally high levels of a certain type of white blood cell, the eosinophil, in the bloodstream. Hypereosinophilia can result in:
•    Fever
•    Night sweats
•    Fatigue
•    Weight loss
•    Coughing
•    Abdominal pain
•    Sometimes gastrointestinal bleeding

The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple parts of the body, including:
•    Skin
•    Heart
•    Nerves
•    Muscles
•    Bones
•    Gastrointestinal tract

Many possible symptoms include:
•    Weight loss
•    Fatigue
•    Joint pains
•    Rashes
•    Numbness
•    Tingling in hands or feet
•    Abdominal pain
•    Cough
•    Chest pain
•    Shortness of breath

The diagnosis of CSS/EGPA is based on a patient’s history in addition to abnormal blood tests and chest x-rays or CT scans. Biopsies may be required of the affected organ.


Treatment & Management

CSS/EGPA is a serious disease that can be fatal without treatment. However, with treatment, CSS/EGPA can often be controlled and complete remission is possible. Treatment typically includes steroids or other immune suppressing medications. Some newer medications called biologics have also been used to treat this disorder.

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