Churg-Strauss syndrome is a medical condition that causes your blood vessels to become inflamed. It’s a form of vasculitis. The condition may also be called eosinophilic granulomatosis with polyangiitis, or EGPA.
Inflammation in your blood vessels makes them narrower and decreases the amount of blood that is able to flow through them. This means that the blood flow to your major organs and systems is also less than normal. A reduced blood flow to your organs may damage them. This organ damage is not always reversible and may be permanent.
What are the symptoms?
The symptoms of Churg-Strauss syndrome are determined by what organs or systems the condition effects. Some of these symptoms may include:
- extreme tiredness
- shortness of breath, caused by inflammation of the lung air sacs or the blood vessels
- chest pain, caused by inflammation of the lungs or heart
- numbness in hands or feet
- pain in the abdomen
- blood in your stools
- sinus pain or a runny nose
- joint pain
- muscle pain
- skin rash
- loss of weight
- night sweats
- kidney disease
You may have just some of these symptoms and not all of them. You also could have combinations of any of these symptoms.
What are the causes?
It’s not clear what causes Churg-Strauss syndrome. However, asthma seems to be a common denominator in people with the condition. There have been studies that have looked at whether or not one of the ingredients in a common medication for severe asthma, Montelukast, may cause or trigger it.
So far, there is not enough evidence to show that Montelukast causes Churg-Strauss. However, there is evidence indicating that Montelukast may trigger Churg-Strauss if It’s already a previously undetected condition.
It’s known that Churg-Strauss syndrome is not genetic and is not contagious. It’s also known that an autoimmune condition is involved in some way in the cause of this condition.
Life expectancy and prognosis
The prognosis is usually good if your condition is properly diagnosed and you’re treated with corticosteroids. 90% or more of people that are treated with corticosteroids alone will go into remission and not need any additional treatment.
Relapses are possible, so continued checkups with a medical professional are important. This will ensure that you’ll be able to treat the relapse quickly. Many people will continue to need treatment of asthma even after remission.
If Churg-Strauss syndrome is caught and treated before any major organ damage has occurred, you can live a fairly normal life. If organ damage has occurred, your future prognosis will be determined by the severity of the damage, and how well it responds to treatment.
How is it diagnosed?
The symptoms of Churg-Strauss syndrome can look like a number of other illnesses and medical conditions. Therefore, you doctor may run various tests to rule out other diagnoses. Once other conditions have been ruled out, your doctor may perform additional diagnostic tests to confirm, as well as to discover, which systems are being affected.
Some diagnostic tests may include:
- chest X-rays
- CT scan
- blood tests
To receive a diagnosis of Churg-Strauss syndrome, you normally must have the following six criteria:
- eosinophilia or a high number of white blood cells in your blood
- damage to groups of nerves (either one or several, also called mononeuropathy or polyneuropathy)
- lesions on your chest X-ray that move, also called nonfixed pulmonary infiltrates
- sinus issues
- extravascular eosinophilia or white blood cells outside the blood vessels
Treating and managing symptoms
The first line of treatment is to take corticosteroids, such as prednisone. These may initially be given in large doses, and will eventually reduce to a smaller dose.
If your case is more severe, or the corticosteroids do not provide remission of Churg-Strauss, then immunosuppressant drugs may be given in addition to the corticosteroids.
Examples of immunosuppressant drugs include:
Most of the medications used in the treatment of Churg-Strauss syndrome have side effects that you should be aware of. Some of these side effects can be serious. However, there are things you can do to manage and reduce the effect of them. Some ongoing lifestyle and disease management steps you should take include:
- start or maintain a healthy diet
- stop smoking
- schedule regular checkups with your doctor
- start or maintain an exercise routine after checking with your doctor first
- be sure to maintain enough calcium and vitamin D in your diet, as directed by your doctor, to keep your bones healthy
Complications and outlook
The main complication of Churg-Strauss syndrome is the damage it can do to your organs. This damage can lead to serious medical conditions like:
- kidney disease or failure, this is not as common as other complications
- damage to your peripheral nerves throughout your body
- scars on your skin from rashes or sores that may occur
- damage to your heart which causes various types of heart disease
It’s important to have your doctor thoroughly check you out if you think you may have symptoms that sound like Churg-Strauss syndrome. Your doctor will determine if that is what is causing your symptoms, or if you have another medical condition. Once diagnosed, your doctor will be able to give you an effective treatment plan.