Churg-Strauss Syndrome (CSS), or Allergic Granulomatosis, is a rare autoimmune disease with no known cure. It involves an over active response where the immune system targets healthy tissue, causing widespread inflammation. Small arteries and veins in the lungs, skin, nerves and abdomen become inflamed, and blood flow is restricted, causing temporary or permanent damage to various organ systems. Churg-Strauss Syndrome mostly occurs in people with a history of asthma or allergies that are hard to control. Effects vary widely from patient to patient. The average age at diagnosis ranges from 35 to 45 and the disease affects slightly more men than women.
CSS is difficult to diagnose as its symptoms are similar to those of various other conditions. The most typical symptoms of CSS include asthma (being the most common sign of CSS), sinus problems, numbness or pain in one's extremities, gastrointestinal bleeding, fatigue, shortness of breath, chest pain, rashes, diarrhoea and irregular heartbeat.
The exact causes of CSS remain unknown. It is speculated that genetics, as well as exposure to industrial solvents, may predispose one towards the disease. However, this remains largely unproven. Although no link between asthma medication and CSS has been proven, some patients have developed CSS after using asthma and allergy medication.
Owing to the difficulty of diagnosing CSS, many people are diagnosed in the late stages of the disease. The American College of Rheumatology has identified six criteria relevant to CSS. The presence of two or three of these criteria may lead a doctor to diagnose CSS. The criteria are:
Doctors may use one of several diagnostic tests including blood tests (to detect autoantibodies in the blood stream), imaging tests (x-rays, CT scans and MRI scans of the lungs and sinuses), and biopsies of affected tissue.
Treatment is very effective when the disease is caught in the early stages. High doses of oral steroids are usually administered to get the disease under control as soon as possible. Once improvement is obvious, the dose is then slowly lowered to a maintenance dose. For those people who have life threatening complications or those who do not respond to the steroids, other immunosuppressant drugs may be prescribed.
Whilst there is no cure for CSS, remission is possible. Life expectancy of people with CSS has risen substantially since it was discovered in 1951. Most people will remain on medication for life and relapses are common, however, with early diagnosis most people's life expectancy runs into many years.