Cease Churg-Strauss Syndrome

You have shortness of breath. You experience chest pain as well as muscle and joint pain. You have a rash. You could have Churg-Strauss syndrome.

Churg-Strauss syndrome is both a pulmonary disorder as well as a form of vasculitis (a blood-vessel inflammation). Asthma is the most common sign of the condition. If left untreated, Churg-Strauss can destroy organs.

In Churg-Strauss syndrome, certain cells and tissue inflame. The inflammation can affect different organs like the heart and kidneys. Although the cause of the inflammation is unknown, some doctors think that it is the immune system not functioning properly.

Another name for the condition is eosinophilic granulomatosis with polyangiitis or EPGA. Anyone diagnosed with Churg-Strauss syndrome usually has a history of asthma or allergies. According to the Cleveland Clinic, almost all people with Churg-Strauss have a number of “allergic type” blood cells referred to as eosinophils. Eosinophils are a type of white blood cell that make up a small percentage of the total white blood cell count. In Churg-Strauss, eosinophils make up more than 10 percent of the total white blood cell count.

Vasculitis means inflammation of the blood vessels. Some things that can happen as a result of vasculitis are:

·       Your blood vessels can grow weak and stretch, which can cause an aneurysm to develop. An aneurysm is an abnormal blood filled enlargement of a blood vessel.

·       The walls can grow thinner. When that happens, the walls can rupture and blood can leak out into tissue.

·       Blood vessels can narrow to the point of closing. This can cause damage to the organs from the loss of nutrients and oxygen that were being supplied by blood.

The inflammation in question can cause damage to your lungs, joints, skin, nerves, nose, sinuses, intestinal tract, heart and/or kidneys and in rare instances, the brain.

Churg-Strauss syndrome is an extremely rare condition. Churg-Strauss can affect anyone of any age. Diagnosis usually happens from age 35 to 50 years old. The condition affects men and women equally. Churg-Strauss syndrome isn’t contagious.

While the cause of inflammation in Churg-Strauss isn’t known, your immune system plays an important role in the condition. Your immune system usually works to protect the body from germs and bacteria that can cause disease, infections and injuries to the body. But sometimes the immune system can overreact and cause inflammation.

Because Churg-Strauss can affect a wide range of organs, there are different kinds of symptoms.

People with the condition can feel ill, fatigued or have fever. They can lose their appetite and lose weight. Other symptoms depend on the organ affected. You can have:

·       Shortness of breath from asthma or inflammation in the air sacs and blood vessels of the lungs.

·       Chest pain from disease that affects the lungs or heart.

·       Skin rashes.

·       Muscle and/or joint pain.

·       Greater nasal discharge or facial pain from sinusitis.

·       Abdominal pain or blood in the stools from intestinal tract involvement.

·       Unusual feelings, and numbness or loss of strength and feeling from nerve involvement.

Often, kidney disease caused by Churg-Strauss doesn’t have any symptoms. You may not notice anything until the kidneys stop working. You may have to have regular urinalyses (examinations of the urine).

To diagnose Churg-Strauss, your health provider may use tests like medical history, physical examination, blood tests, urinalysis and imaging tests such as computed tomography (CT) and X-rays. In addition, a biopsy (tissue sample) is performed to find eosinophils, eosinophilic granulomas and/or vasculitis. Biopsies are needed when unusual results are found in the exam, laboratory tests or imaging tests.

To treat Churg-Strauss syndrome, different kinds of immunosuppressive drugs (drugs that suppress the immune system) are used. The drug will be chosen by your doctor based on how the condition is affecting you, which can range from mild to life-threatening.

Glucocorticoids including prednisone or prednisolone are the most popular treatments. For milder treatment of the disease, corticosteroid therapy will suffice. Once you begin to recover while taking the drug, the dosage is reduced to the lowest amount possible to keep the disease under control.

People with Churg-Strauss affected by critical organs are usually treated with corticosteroids combined with immunosuppressive drugs like cyclophosphamide (Cytoxan), methotrexate, Mycophenolate Mofetil or azathioprine (Imuran).

These medications may be used to treat other medical conditions. For instance, azathioprine is used to prevent organ transplant rejection as well as lupus and other autoimmune diseases. Increased doses of cyclophosphamide and methotrexate are used to treat certain types of cancer. In vasculitis, these drugs are prescribed for 10 to 100 times lower than that used to treat cancer in order to suppress the immune system.

Biologic medications are also used to treat Churg-Strauss such as Mepolizumab (anti-interleukin (IL)-5 monoclonal antibodies and Rituximab (anti-CD20 monoclonal antibody directed against B cells).

The aim of treatment is remission or to stop all damage. Once the condition has improved, your health provider may lower the corticosteroid dose and eventually stop it altogether. Stopping it altogether may be difficult for a person with asthma, as the airways can be sensitized to corticosteroids.

Churg-Strauss is an illness that affects a plethora of organs but a majority of patients survive the symptoms. Be sure to get treated early and follow your health provider’s advice.