What Is Churg Strauss Syndrome

Churg- Strauss Syndrome is also known as Ďallergic granulomatosis, which is characterized by vasculitis (i.e. inflammation of blood vessels), due to autoimmune causes. This condition affects small and medium blood vessels, especially of the lungs, heart, skin and kidneys along with peripheral vascular system.

Churg- Strauss Syndrome: An Overview

The condition is a rare non communicable disorder and was first described by the doctor duo in 1951. The American college of Rheumatology has identified six basic criterions to diagnose Churg- Strauss Syndrome. The presence of four or more of the criteria is considered adequate to diagnose the condition. These criteria include asthma (characterized by ronchi and wheezing), eosinophilia (characterized by elevation of eosinophil count in the blood to 10% to total WBC count), sinusitis, pulmonary infiltrates, histopathological evidence of vasculitis and polyneuropathy or mononeuritis multiplex.

The incidence of Churg- Strauss Syndrome is 1 to 3 per 100000 adults per annum in the United States, while internationally the incidence is at approximately 2.5 per 100000 adults per annum. The condition tends to affects males more than females and with treatment the one year survival rate is as high as 90% while five years survival rate stands at 62%. Churg- Strauss syndrome may start any time between 15-70 years but on an average the mean age of diagnosis is approximately 50 years.

Diagnosis, Prognosis and Treatment of Churg- Strauss Syndrome

The prognosis of the condition depends upon the severity of the disease. As per the French Vasculitis Group, a five point score has been developed to understand the risk of death associated with Churg-Strauss Syndrome. Presence of any one of the indicators (i.e. reduced renal function, proteinuria, gastrointestinal hemorrhage, cardiomyopathy and involvement of the nervous system) have been associated with five year mortality of 26% while presence of any two of the parameters indicate a higher five year mortality rate of 46%. The risk of five year mortality reduces when none of the indicator symptoms are present to as low as 11.9%.

A detailed medical history of the patient is crucial in determining the presence of Churg-Strauss Syndrome. Abnormalities of the lungs, skin, and nerves with elevated blood pressure are indicative of the condition. Blood examination can help identify eosinophilia, while urine analysis can help diagnose abnormalities in the urine. Chest X ray or CT scan images can help identify the areas of inflammation in the lungs.

Microscopic evaluation of the lung tissue to observe a characteristic inflammation pattern is indicative of Churg- Strauss Syndrome.