What Is CSS

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The initials CSS stand for…

Churge Strauss Syndrome

An explanation about CSS is defined below, as taken from the Churge Strauss Syndrome Association website;

What is Churg Strauss Syndrome?

Churg Strauss Syndrome is a rare systemic autoimmune disease characterized by inflammation of small to medium sized arteries, arterioles and venules. This inflammatory process of blood vessels is more commonly known as vasculitis. Churg Strauss vasculitis is characterized by the invasion and abnormal increase of a white blood cell known as an eosinophil. The eosinophils cluster together and release harmful granules that collect in different parts of the body as inflammatory nodule lesions. This is called granulomatosis. This eosinophilic inflammation, along with asthma, are the hallmarks of Churg Strauss Syndrome. The inflammatory process can cause impaired blood flow to various organ systems. The resultant damage to different organs may be temporary or permanent.

Churg Strauss Syndrome should be considered when there is late onset asthma, or worsening asthma, along with either numbness or pain in the extremities, sinus problems, a lingering cough, a rash, stomach problems, or symptoms of cardiac involvement.

The American College of Rheumatology established the following criteria for diagnosing CSS:

Asthma
Eosinophilia >10%
Neuropathy, mono or poly
Pulmonary infiltrates, non-fixed
Paranasal sinus abnormality
Extravascular eosinophils

For classification purposes, a patient is said to have Churg Strauss syndrome if at least 4 of these 6 criteria are positive. Some researchers believe that early cases of CSS may consist of asthma and tissue eosinophilia without detectable vasculitis. If caught early, CSS is more responsive to corticosteroids, while cases with full-blown vasculitis may require the addition of more powerful immunosuppressive drugs. Unfortunately, early stage CSS is often under-diagnosed.

Churg Strauss is a baffling disorder that is difficult to diagnose and one whose effects vary widely from patient to patient. Some people have mild symptoms which barely affect day to day living while others suffer from a wide variety of problems including sinus problems, rashes, lung involvement, peripheral neuropathy, gastrointestinal problems and heart involvement. CSS is not contagious and is not inherited. Its cause is unknown. There is no cure, but many people achieve long term remissions. It affects men slightly more than women. It can affect people of all ages with the average age at diagnosis being 35 to 45. Estimates about the incidence of CSS vary widely and range from 2.4 to 10 cases per 1 million people, or roughly from 720 to 3,000 people in the United States.

CSS was almost always a fatal disease until the discovery of effective drug therapy. Treatment consists of quieting the inflammation of the blood vessels and suppressing the immune system. Corticosteroids are usually the initial therapy. For those with more severe, life threatening complications, or those who fail to respond to steroids alone, cytotoxic drugs may be added. Side effects and drug toxicity need to be carefully monitored during treatment. The chief causes of mortality are severe asthma, cardiopulmonary failure, or gastrointestinal complications. With prompt diagnosis and treatment many people achieve at least medically maintained remission, although some may experience occasional flares.

Because there is no cure for CSS and relapses, or flares, are common it is very important that the disease be carefully monitored by a physician with regularly scheduled lab tests even while in remission. CSS may present differently during a flare than when first diagnosed, so any new symptoms should be reported promptly. CSS is a chronic and sometimes life threatening disease, but with diligence and effective medical care most patients enjoy a very good quality of life.

This is the basic definition of the disease.