Churg-Strauss syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at April 29, 2016
StartDiseasesChurg-Strauss syndrome

Churg-Strauss syndrome is a rare autoimmune disease and a form of vasculitis, which is characterized by inflammation of blood vessels. It is mostly found in patients that have a history of asthma or lung disease. It is a fatal disease if left untreated and known treatment options cause many side effects. Churg-Strauss is also known as eosinophilic granulomatosis with polyangiitis.

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Definition & Facts

Churg-Strauss causes inflammation of blood vessels, such as small arteries and veins, in the lungs, skin, and abdomen. This inflammation can restrict blood flow to major organs and tissues within the body.

Over time, the lack of blood flow can permanently damage most bodily organs. It occurs mostly in people with asthma or allergies that affect breathing. It causes those who are affected to experience extreme fatigue and weakness. A rare disorder, research has found that around 3,000 Americans have Churg-Strauss syndrome.

Symptoms & Complaints

Some symptoms of Churg-Strauss syndrome include fever and unexplained weight loss. Other symptoms consist of fatigue, shortness of breath, and chest pain. As the disease progresses, symptoms worsen. Other physical symptoms of Churg-Strauss include gastrointestinal bleeding and severe pain and numbness in extremities such as hands and feet.

If the brain or central nervous system is affected by Churg-Strauss syndrome, the patient may experience seizures, confusion, and motor problems. Additional symptoms of Churg-Strauss include diarrhea and skin lumps (also known as nodules). Churg-Strauss causes significant organ damage, and without treatment it will progress until it leads to organ failure; heart failure is the leading cause of death for those suffering from this disease.

Causes

The immediate cause of Churg-Strauss is relatively unknown since it is so rare. Research has been unable to find a definitive genetic cause, and incidences of the disease occurring within the same family are rare. What is known is that Churg-Strauss syndrome involves an overreaction of the immune system and that it tends to affect people with a history of asthma or allergic rhinitis. The overreaction of the immune system means that it attacks healthy tissue instead of viruses and harmful bacteria in the body.

People with a history of sinus problems are also at risk for Churg-Strauss syndrome. One theory for the cause of Churg-Strauss is the use of leukotriene modifiers that are present in some asthma medications. This theory is not fully accepted since it is unknown if the cause is from the medication or the severity of the asthma in the patient.

Diagnosis & Tests

Churg-Strauss is usually diagnosed when a doctor detects high blood pressure; abnormalities of the skin, nerves, and lungs are present; and the patient is in their late 40's and has a history of asthma. Though the syndrome is seen among a wide age range – from the teens into the late 70's - it is more commonly diagnosed in people that are around fifty years old.

Blood tests of people with Churg-Strauss syndrome will typically show elevated levels of eosinophil, an uncommon white blood cell. Eosinophil usually accounts for 1 to 3 percent of white blood cells; however it is elevated to over 10% in those with Churg-Strauss syndrome. Pulmonary infiltrates are also found on chest X-rays of the patient. Eosinophil release harmful granules, which collect in different regions of the body as inflammatory nodule lesions in a process that is called granulomatosis.

The primary test for diagnosing Churg-Strauss is a biopsy of the affected skin tissue. The tissue will show patterns of inflammation when viewed under a microscope and the tissue will also contain traces of eosinophil. Most people diagnosed with Churg-Strauss syndrome have a type of nerve damage called peripheral neuropathy. This nerve damage causes numbness or pain in the hands and feet of the patient.

Treatment & Therapy

Treatment of Churg-Strauss starts immediately after the patient is diagnosed with the syndrome. The treatment involves lessening the inflammation of the tissues and suppressing the immune system. This treatment method involves medication with high levels of cortisone, such as prednisone or prednisolone, to lower the inflammation and medications with high levels of cyclophosphamide (Cytoxan®), azathioprine (Azasan®, Imuran®) or methotrexate (Rheumatrex®, Trexall®) to suppress the immune system.

Medications with cyclophosphamide are usually given for at least one year. New studies indicate that there is a way for doctors to give versions of Cytoxan® with lower levels of cyclophosphamide to patients for less than six months. This new form of Cytoxan® will be less toxic for the patients.

Those who do not respond to other forms of treatment are typically given monthly infusions of immune globulin. That is not a widely used form of treatment since it is extremely expensive and does not work for everyone. Some drugs that have gone through experimental tests on patients include rituximab; these drugs have been shown to reduce symptoms of Churg-Strauss and decrease the count of eosinophil in the body.

Prevention & Prophylaxis

There is no known way to prevent Churg-Strauss syndrome. It is so rare that it is not commonly studied among researchers. For now, main suggestions from doctors and researchers include the avoidance or cessation of smoking and maintaining a healthy lifestyle along with regular exercise and a healthy intake of vitamin D.

Since one part of the treatment of Churg-Strauss syndrome is the suppression of the immune system, many patients diagnosed with Churg-Strauss syndrome are given antibiotic prophylaxis along with their other medications. The antibiotics will be prescribed to prevent infections usually to people following surgery or those with weakened immune systems.