What is the Churg-Strauss syndrome?

Churg-Strauss Syndrome is a very rare, life threatening autoimmune disease involving inflammation of the blood vessels, or vasculitis.

It develops when a type of white blood cell called an eosinophil invades blood vessel walls and different body organs and causes inflammation. The usual organs involved are the lungs, sinuses, peripheral nerves (such as the ones that control the arms and legs) skin, heart, stomach, intestinal tract and occasionally the brain, kidneys or central nervous system.

Churg-Strauss Syndrome is not contagious. It is not an inherited disease. No one knows what causes it.

What are the symptoms of Churg Strauss Disease?

The symptoms of the Churg Strauss Syndrome are multiple and depend on the progress of the disease. More often several of the following symptoms occur together:

• Newly developed asthma, or worsening of asthma
• History of allergy
• Chronic rhinitis and sinusitis
• Fatigue
• Unexplained weight loss
• Rashes
• Peripheral neuropathy, or unusual sensation in the limbs
• Heart affections, pericarditis
• Stomach pain, gastrointestinal problems

What is an auto-immune disease?

An auto-immune disease is a disorder of the immune system causing it to attack its own body.

What is a vasculitis?

Vasculitis is simply an inflammation of the blood vessels or blood vessel wall. It can range from mild to severe where the blood vessel can actually necrose or (die off) in parts.There are many different reasons for vasculitis besides CSS.

What is the treatment?

The treatment of CSS is to bring the inflammation of the blood vessels and organs under control. This is done by initiating treatment with prednisone or steroid medication. Depending on the severity of the disease a second immunosupressent drug such as Cytoxin or Imuran may be needed. As the inflammation subsides the medications can be tapered.

What is the prognosis for patients with Churg Strauss Syndrome?

Unfortunately, there is no cure for patients with Churg Strauss Syndrome, although remission is possible. A good quality of life may be achieved thanks to the progress of medical research. Life expectancy with Churg-Strauss Syndrome has increased dramatically since it was discovered in 1951, from some months to many years. Occasionally the damage to the heart, kidneys or nervous system remains even when the disease is in remission.

What is a flare?

A flare is a new occurence of the Churg-Strauss symptoms during the remission period. It must be detected and treated rapidly.


For more information see More about Churg-Strauss syndrome