Churg-Strauss syndrome (CSS), also known as allergic granulomatosis, is a rare autoimmune disease that causes vasculitis (blood vessel inflammation), which restricts blood flow to various organs, especially the lungs and skin. The restricted blood flow to these organs can cause temporary or permanent damage.
Two scientists, Jacob Churg and Lotte Strauss, first described CSS in 1951 when they reviewed autopsy cases that were previously classified as polyarteritis nodosa (disease that causes inflammation of the arteries). These cases were unusual because they were associated with asthma and extravascular granulomas (clumps of cells that form lumps on the outside of blood vessels), as well as a systemic vasculitis.
Individuals who have CSS have an increased level of white blood cells, known as eosinophils. The eosinophils cluster together and release harmful granules, which collect in different regions of the body as inflammatory nodule lesions. This process is called granulomatosis.
The age at onset (when symptoms first arise) varies from 15-70 years, with an average of about 38 years. The average age at diagnosis is about 50 years. Researchers estimate that anywhere between 720 and 3,000 Americans have CSS. For unknown reasons, CSS afflicts slightly more men than women, and it can affect individuals of all ages.
CSS is not contagious or hereditary. While there is no cure, many patients achieve long-term remissions. CSS fatalities are usually caused by severe asthma, cardiopulmonary (heart and lung) failure or gastrointestinal complications. With treatment, the one-year survival rate after diagnosis is about 90% and the five-year survival rate is about 62%.
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