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What is Churg-Strauss Syndrome?

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  • Written By: Mary McMahon
  • Edited By: O. Wallace
  • Images By: Alexander Gospodinov, Thirteen Of Clubs
  • Last Modified Date: 30 August 2016
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Churg-Strauss syndrome is a rare autoimmune disorder first described in 1951. It is a form of vasculitis, in which the blood vessels become inflamed, with the inflammation spreading to organ systems such as the lungs and skin. Historically, this condition was fatal, because the body was not able to cope with the inflammation. Modern treatments have made Churg-Strauss syndrome manageable, especially if it is caught early, before the patient has experienced organ damage.

This condition usually starts with the onset of allergic rhinitis, nasal polyps, and sinus irritation. Eventually, the patient develops asthma, which becomes more severe over time as Churg-Strauss syndrome gradually moves into the third phase, which involves damage to the body's organ systems. If blood samples are taken from the patient, they reveal a high concentration of a type of white blood cell called an eosinophil. Normally, these cells are part of the immune system, but when they are present in large numbers, they cause inflammation.

Symptoms of Churg-Strauss syndrome can include shortness of breath, nausea, vomiting, diarrhea, weight loss, skin problems, and abdominal pain. Churg-Strauss can also involve the nerves, causing tingling, numbness, and pain. A doctor can use a combination of blood work, patient history, and medical imaging studies to diagnose a patient with the condition and determine the extent of the damage. Patients may hear Churg-Strauss syndrome referred to as allergic angiitis or allergic granulomatosis.

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The treatment for this condition involves high doses of prednisone to address the inflammation, with dosage gradually being tapered down over time. Immunosuppressive drugs may also be used to blunt the immune system response. Treatment can take one to two years, with the patient being carefully monitored during the treatment to confirm that the dosages of medications are appropriate and to look for signs of damage. Patients usually see a rheumatologist for Churg-Strauss syndrome treatment.

As with many autoimmune conditions, the cause of Churg-Strauss syndrome is not known. There does not appear to be a genetic component, and the condition is not communicable, as it involves a fundamental problem with the patient's immune system, rather than a disease-causing agent which could be passed on. Men and women appear to be at equal risk of developing this condition, and the average age of onset is around middle age. Patients can sometimes live for years in the early stages of the syndrome, and it can be tricky to diagnose because the symptoms are often vague and very broad in the early stages. The rarity of the condition also means that a doctor will be less likely to suspect Churg-Strauss until the syndrome has progressed quite far.

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