Churg-Strauss Syndrome (CSS), or Allergic Granulomatosis Angiitis

Churg-Strauss syndrome is one of many forms of vasculitis, which is the inflammation of blood vessels. Churg-Strauss syndrome, in particular, occurs in patients with a history of asthma or allergies and features inflammation of blood vessels in the lungs, skin, nerves, and abdomen. The blood vessels involved in CSS are usually the small arteries and veins. The syndrome is rare and has no cure. The cause of CSS is not known, but it involves an abnormal over-activation of the immune system in a person with underlying lung disease (asthma).

Churg-Strauss syndrome causes fever, weight loss, fatigue and sinus or nasal passage inflammation in the patient with asthma. Cough, shortness of breath, and chest pain can occur as the lungs are affected by vasculitis. Skin lumps, called nodules, can appear on the extremities and the bladder and prostate gland can become inflamed.

The treatment of patients with Churg-Strauss syndrome is directed toward both immediately quieting the inflammation of the blood vessels (vasculitis) and suppressing the immune system. Treatment usually includes high doses of cortisone-related medication such as prednisone to calm the inflammation and suppression of the active immune system with cyclophosphamide.

Prior to the advent of prednisone, CSS was often a fatal disease. The majority of patients died from a rampant, uncontrolled disease. However, now with present therapy, the disease is controlled for many patients.


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