By Doc Willie Ong
Churg-Strauss syndrome is a blood vessel inflammatory illness. It is a rare systemic necrotizing vasculitis that affects small-to-medium-sized arteries and is linked with severe asthma, blood and tissue eosinophilia. This inflammation can reduce blood supply to organs and tissues, potentially causing irreversible damage. Eosinophilic granulomatosis with polyangiitis is another name for this disorder (EGPA).
It is an uncommon disease that affects about 11 to 14 people per million people around the world. While people of various ages can develop the condition, the average onset age is 38 to 54 years old.
Unlike other vasculitides, Churg-Strauss syndrome is extremely rare in youngsters. Children with Churg-Strauss, on the other hand, usually have a more aggressive course that includes the lungs and cardiovascular system. Children with Churg-Strauss have significantly higher mortality rates than adults.
What causes Churg-Strauss syndrome?
Churg-Strauss syndrome mostly has an unknown cause. An overactive immune system is most likely triggered by a mix of genetics and environmental factors, such as allergens or certain drugs. Rather than fighting invading germs and viruses, the immune system attacks healthy tissue, resulting in extensive inflammation.
How does Churg-Strauss syndrome manifest itself?
It manifests itself differently in each individual. Some people merely experience minor symptoms. Others are dealing with serious or life-threatening situations.
The EGPA syndrome takes place in three stages and gets worse over time. Symptoms of asthma, such as shortness of breath, wheezing, and coughing, as well as symptoms of rhinosinusitis, such as facial pain and nasal congestion, are common in Churg-Strauss syndrome patients.
Other signs and symptoms to look out for are:
- Appetite loss and weight loss
- Muscle and joint pain
- Gastrointestinal bleeding and abdominal pain
- Weakness, weariness, or an overall sense of ill health
- Rash or blisters on the skin
- In your hands and feet, you may experience pain, numbness, and tingling.
What is the treatment for Churg-Strauss syndrome?
Although there is no treatment for Churg-Strauss syndrome, patients should seek medical guidance to acquire medicines that will help them manage their symptoms and reduce inflammation.
Glucocorticoids in medium to high doses are commonly used in the early stages of treatment (e.g., prednisone). Inflammation and eosinophil levels in tissues and blood vessels are reduced by glucocorticoids.
After achieving remission, the dose of glucocorticoids is usually gradually reduced to minimize acute and long-term side effects. In majority of cases, they are eventually phased out.
Individuals can be given additional immunosuppressant drugs, such as azathioprine, methotrexate, or cyclophosphamide, if glucocorticoid-related toxicity arises or the condition remains uncontrolled on glucocorticoids.
What are the complications of Churg-Strauss syndrome?
Without treatment, the Churg-Strauss syndrome can affect many organs, including the:
- Gastrointestinal system
- Joints and
Complications, which depend on the organs involved, can include:
Peripheral nerve damage.
It can damage the nerves in your hands and feet, leading to numbness, burning and loss of function.
Heart-related complications of Churg-Strauss syndrome include inflammation of the membrane surrounding your heart, inflammation of the muscular layer of your heart wall, heart attack and heart failure.
If Churg-Strauss syndrome affects your kidneys, you can develop glomerulonephritis. This disease hampers your kidneys’ filtering ability, leading to a buildup of waste products in your bloodstream.