What is Churg-Strauss syndrome?
Churg-Strauss syndrome (CSS) is an extremely rare
disease – there are only 2 to 5 new cases per year per 1 million people. CSS
results from inflammation that occurs in certain types of cells in blood or in
tissues. This inflammation causes injury to organ systems – the most commonly
involved are the lungs, nose, sinuses, skin, joints, nerves, intestinal tract,
heart, and kidneys. A main feature of CSS is that all patients have had a
history of asthma and/or allergies.
Almost all people with CSS have increased numbers of
"allergic type" blood cells called eosinophils. Eosinophils are a type of white
blood cell. Normally, eosinophils make up 5% or less of the total white blood
cell count. In CSS, eosinophils usually make up more than 10% of the total white
blood cell count. In addition, most biopsies contain clusters of cells called "granulomas"
that may or may not involve blood vessels. The granulomas often include
eosinophils and are therefore called "allergic granulomas." In fact, another
name for CSS is allergic granulomatosis. Some of the most serious manifestations
of CSS are related to the presence of vasculitis.
What is vasculitis?
Vasculitis is a general medical term that refers to
inflammation of the blood vessels. When blood vessels become inflamed, they can
stretch, become thin-walled, or narrow in size. When they weaken and stretch in
size, aneurysms can develop. (An aneurysm is an abnormal blood-filled expansion
of a blood vessel.) When they thin, the walls can rupture and blood leaks into
tissue. Vasculitis can also cause blood vessels to narrow to the point of
closing. Organs can be damaged from the loss of oxygen and nutrients that were
being supplied by the blood.
What are the symptoms of CSS?
Because many different organ systems may be involved,
a wide range of symptoms are possible in CSS.
Patients who have CSS may feel generally ill and
fatigued, have fevers, or have a loss of appetite and weight. Other specific
symptoms depend on the organs or diseases involved. For example, patients may have:
- shortness of breath from asthma or inflammation in the air sacs and
blood vessels of the lung
- chest pain from disease affecting the lungs or heart
- rashes on the skin
- muscle and/or joint pain
- increased nasal discharge or facial pain from sinusitis
- abdominal pain or blood in the stools occurring as a result of
intestinal tract involvement
- abnormal sensations followed by numbness or loss of strength and feeling
as a result of nerve involvement