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Churg-Strauss syndrome is a rare autoimmune disease causing inflammation of blood vessels, which can spread to organs such as the lungs and skin. Symptoms include shortness of breath, skin problems, and abdominal pain. Treatment involves high doses of prednisone and immunosuppressive drugs. The cause is unknown, and it can be difficult to diagnose in the early stages.
Churg-Strauss syndrome is a rare autoimmune disease first described in 1951. It is a form of vasculitis, in which blood vessels become inflamed, with the inflammation spreading to organ systems such as the lungs and skin . Historically, this condition was fatal, because the body was unable to cope with the inflammation. Modern treatments have made Churg-Strauss syndrome manageable, especially if it is caught early, before the patient has suffered any organ damage.
This condition usually begins with the onset of allergic rhinitis, nasal polyps, and sinus irritation. Eventually, the patient develops asthma, which becomes more severe over time as Churg-Strauss syndrome gradually progresses to stage three, which involves damage to the body’s organ systems. If blood samples are taken from the patient, they reveal a high concentration of a type of white blood cell called an eosinophil. Normally, these cells are part of the immune system, but when they are present in large numbers they cause inflammation.
Symptoms of Churg-Strauss syndrome can include shortness of breath, nausea, vomiting, diarrhea, weight loss, skin problems, and abdominal pain. Churg-Strauss can also involve nerves, causing tingling, numbness, and pain. A doctor can use a combination of blood tests, patient history, and medical imaging studies to diagnose a patient with the condition and determine the extent of the damage. Patients may hear Churg-Strauss syndrome referred to as allergic angiitis or allergic granulomatosis.
Treatment for this condition involves high doses of prednisone to address the inflammation, with the dosage being gradually reduced over time. Immunosuppressive drugs can also be used to blunt the immune system response. Treatment can take one to two years, with the patient closely monitored throughout treatment to confirm that drug dosages are appropriate and to look for signs of damage. Patients usually see a rheumatologist for treatment of Churg-Strauss syndrome.
As with many autoimmune conditions, the cause of Churg-Strauss syndrome is not known. There does not appear to be a genetic component and the condition is not transmissible, as it involves a fundamental problem with the patient’s immune system, rather than a pathogen that could be passed on. Men and women appear to be equally at risk of developing this condition, and the average age of onset is around middle age. Patients can sometimes live for years in the early stages of the syndrome and it can be difficult to diagnose because symptoms are often vague and very broad in the early stages. The rarity of the condition also means that a doctor will be less likely to suspect Churg-Strauss until the syndrome has progressed far enough.
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