Churg Strauss syndrome is a type of vasculitis (blood vessel inflammation) that occurs throughout the body. The American College of Rheumatology defined criteria for a diagnosis of Churg Strauss syndrome:
- Asthma
- High numbers of eosinophils, a type of white blood cell, in the blood
- Pain, numbness, or tingling in the arms and legs (called mononeuropathy)
- Lung abnormalities
- Sinus problems such as abnormal growths (polyps)
- A tissue sample showing a blood vessel with eosinophils around it
Are there other symptoms?
Besides the criteria for diagnosis listed above, the person with Churg Strauss syndrome may have:
- Lumps (nodules) or raised patches (purpura) in the skin
- Kidney, abdominal, or heart problems because of blood vessel inflammation
- Weakness and fatigue (malaise)
- Loss of appetite (anorexia) and weight loss
- Other symptoms specific to the part of the body affected in the person
Who gets it?
Usually, a person already has asthma when he or she develops Churg Strauss syndrome. What brings on the syndrome is not yet clear, but research indicates it is an autoimmune disorder, meaning the body's white blood cells, which usually fight off diseases, begin attacking healthy tissue. It affects males and females equally.
How is it treated?
Since the syndrome seems to be caused by a problem with an overactive immune system, medications are given to slow down (suppress) its activity. Prednisone is the medication usually used, but other immunosuppressive drugs such as azathioprine (Imuran), mycophenolate (CellCept), methotrexate, or cyclophosphamide (Cytoxan) may be added if needed. High doses of medication can be given intravenously in severe cases. Symptoms usually start to resolve quickly after starting medication, but treatment can last for 1 or 2 years depending upon how severe the syndrome is in the individual.
What is the future for research?
Discovering what causes Churg Strauss syndrome will help doctors find a way to control it, or even prevent it from occurring. It does not seem to occur in families, so although genetics may play a part in its development, it does not seem to be inherited. It is possible that exposure to environmental toxins may play a role, as it does in other autoimmune disorders. The syndrome may be caused by an overactive immune system that was set in motion by some kind of infection. Some or all of these factors may be involved; it will be up to research to identify the precise cause(s) of the disorder.
Information for this article was taken from:
- Johns Hopkins Vasculitis Center. Churg-Strauss Syndrome.
- National Organization for Rare Disorders. Churg Strauss Syndrome.
