Churg-Strauss Syndrome

Churg-Strauss syndrome is a rare disease that has no cure.  It is a disease that causes blood vessel inflammation.  The inflammation of the blood vessels can cause blood flow to be restricted to organs and tissues.  It can affect multiple organ systems. 

Churg-Strauss syndrome occurs in people who have a history of asthma or allergies.  The inflammation usually affects the nose, sinuses, heart, intestines, nerves, kidneys, muscles, and joints.  It is caused by an overactive immune system.  Churg-Strauss syndrome can affect anyone, but is usually diagnosed in people between age 30 to 50 years old.

3 Stages

There are three main stages of Churg-Strauss syndrome.  Though there are three stages they may not progress by stage.  As well you may not have all three stages. 

The first stage is known as the allergic stage.  It can last months or even years.  It is usually the first signs and symptoms of the syndrome.  Symptoms of this stage are asthma, existing asthma worsening, hay fever, inflamed sinuses, and nasal polyps.

The second stage is Eosinophilic.  This is when your body is producing too many white blood cells.  Symptoms included with this are weight loss, no appetite, fever, night sweats, joint pain, asthma, fatigue, cough, and belly pain. 

The third stage is Vasculitic.  This is when the inflammation narrows the blood vessels.  This causes there to be less blood reaching your body.  Symptoms that could occur are weakness, fatigue, weight loss, swollen lymph nodes, skin sores, rashes, achy or swollen joints, pain, numbness, and tingling in your feet and hands, belly pain, diarrhea, nausea, vomiting, and blood in your urine.

Symptoms 

Symptoms can vary between each person with Churg-Strauss syndrome.  Symptoms can be mild while other people can suffer from severe symptoms.  Usually symptoms get progressively worse over time.  General symptoms of Churg-Strauss syndrome are hay fever, fever, loss of appetite, weight loss, joint or muscle pain, fatigue, cough, abdominal pain, GI bleeds, weakness, fatigue, skin sores, abdominal pain, shortness of breath, and blood in your urine. 

Complications 

This disease can cause some serious complications.  

• It can cause nerve damage.  
• Skin sores or rashes can leave scars.  
• Pericarditis is another complication which is when the membranes around your heart are inflamed.  
• Myocarditis is another complication that is when the muscles in your heart are inflamed.  
• Kidney problems can also be caused.  This can be a problem that if your kidneys aren’t functioning correctly and not filtering waste, waste can actually end up in your bloodstream. 

Diagnosis

There is no one test to identify Churg-Strauss syndrome.  There are six symptoms that are used as a criteria to start the diagnosis process.  These are; asthma, high white blood cell count, signs of nerve damage, spots or lesions on chest X-ray, sinus trouble, and white blood cells outside blood vessels.  

If you have signs of these six things your doctor may want to do other tests as well.  Blood tests to check for white blood cell counts.  Imaging tests such as X-rays, CT, or an MRI.  Your doctor may want to do a biopsy as well.  This could be a biopsy of your skin, kidneys or lungs. 

Treatment

Though there is no cure for Churg-Strauss syndrome, leaving this untreated can be fatal.  Since there is no cure, treatment is focused on managing symptoms.  

One of the main treatment options is Steroids.  One of the main steroid choices is prednisone.  Once your symptoms are under control your doctor will be able to decrease your dosage of prednisone.  

Immune system drugs are also a choice of treatment.  These drugs will suppress your immune system.  Once under control you may be able to stop taking these, though this could take months or even years before you can stop treatment.  Immune globulin is a monthly infusion.  This is usually done when other treatments aren’t working.  

Biologic medications are also an option.  These alter the immune system’s response. Drug therapy has shown to be able to send this disease into remission though relapses are very common. 

Bone Health

Keeping your bones healthy can also be very important when you have Churg-Strauss syndrome.  Adding in some Vitamin D and calcium supplements can help keep your bones strong.  Some of the treatment medications can cause weight gain.  Making sure you exercise regularly can be very beneficial.  Stopping smoking and eating healthy food are both good things to do. 

Conclusion

People can usually manage their symptoms and live a normal life with Churg-Strauss syndrome.  Though leaving it untreated can be fatal.  This syndrome is progressive leading to worsening symptoms over time.  You will need follow up appointments regularly with your doctor to help track the progression of this disease and make sure the treatment is working to keep your symptoms under control.