Churg-Strauss Syndrome (CSS)高建苑.ppt

Churg-Strauss Syndrome (CSS) First Description Churg-Strauss Syndrome (CSS) is a systemic vasculitis. This disease was first described in 1951 by Dr. Jacob Churg and Dr. Lotte Strauss as a syndrome consisting of “asthma, eosinophilia [an excessive number of eosinophils in the blood], fever, and accompanying vasculitis of various organ systems”. CSS shares many of the clinical and pathological features of polyarteritis nodosa (“PAN”, another type of vasculitis). Churg and Strauss discovered that the presence of granulomas as well as the abundance of eosinophils distinguished this disease from PAN. Another name for Churg-Strauss Syndrome is Allergic granulomatosis. Who gets Churg-Strauss Syndrome (the “typical” patient)? The typical patient with CSS is a middle aged individual with a history of new-onset or newly-worsened asthma. The distribution of the disease among males and females is approximately equal. Classic symptoms and signs of Churg-Strauss Syndrome Asthma is one of the cardinal features of CSS. Asthma symptoms may begin long before the onset of vasculitis – e.g., many years before any other symptoms of CSS arise, and long before the diagnosis of CSS is made. Other early symptoms/signs include nasal polyps and allergic rhinitis. The next phase of the disease is often marked by eosinophilia, the finding of an excessive number of eosinophils in the blood or in tissues. An eosinophil is one subtypes of white blood cell. Normally, eosinophils comprise 5% or less of the total white blood cell count. In CSS, the percentage of eosinophils may reach as high as 60%. In the picture below, the eosinophils are shown by the dark pink stain. The third phase of the illness is a vasculitis, which involves the skin, lungs, nerves, kidneys, and other organs. Particular mention should be made of the frequent devastating involvement of the nerves (called mononeuritis multiplex), which produces severe tingling, numbess, shooting pains, and severe muscle wastin