Myositis – Myositis is an autoimmune disease in which the immune system mistakenly attacks muscle in the body. Autoimmune Myositis is a general terms meaning muscle inflammation but there are several diseases that fall under this umbrella:
- Inclusion Body Myositis
- Juvenile Dermatomyositis (JDM)
- Sporadic Inclusion Body Myositis
Generally, the myositis definition is muscle inflammation and muscle damage. usually occurs in adults aged 40 to 60 or in children aged 5 to 15 years. Women are twice as likely as men to develop it. Black people are 3 to 4 times more likely to develop it than white people.
While the immune system may also cause muscle damage in inclusion body myositis, this may not be cause of this disease. Although myositis is often treatable, these diseases are poorly understood and do not always completely respond to current medications.
Symptoms of Myositis
+ trouble rising from a chair
+ difficulty climbing stairs or lifting arms
+ tired feeling after standing or walking
+ trouble swallowing or breathing
+ muscle pain and soreness that does not resolve after a few weeks
+ known elevations in muscle enzymes by blood tests (CPK or aldolase)
+ Although the inflammatory myopathies affect about 50,000 Americans, often they are not diagnosed correctly. In part, this is because patients with autoimmune myopathies have many of the same symptoms as those with inclusion body myositis, toxic myopathy, or muscular dystrophies, which are inherited forms of muscle disease.
There are a number of blood tests the doctor may choose. The following is a list of some of these. Unless otherwise noted, all of these tests require taking a sample of blood by using a needle. Some blood tests require that you do not eat or drink, except for water, for about eight hours before the test. Because exercise can affect some test results, you might need to limit exercise for several days before the test to avoid falsely high results. Certain medications may also affect the results of these tests, so be sure to check with your doctor if you are taking any type of medications at all, including aspirin or other over-the-counter medications.
Aldolase is an enzyme found especially in the liver and skeletal muscles. When the liver or muscles are damaged, the cells release their contents (including aldolase) into the bloodstream. Since muscle weakness can be caused by problems with either the nerves or the muscles, this test identifies weakness caused by muscular problems. Aldolase will not change when weakness is caused by neurological problems.
Normal ranges may vary slightly based on a particular laboratory’s calibrations. There are also slight differences in normal ranges based on age and gender. Generally, however, normal adult findings are between 1.0 to 7.5 units per liter.
Antinuclear Antibodies (also known as ANA) is a screening blood test to determine if you have an autoimmune disease. Antibodies are an important part of the immune system. They fight infections, viruses, and other foreign substances that can cause disease. When this protective system turns toward fighting the body’s own tissues, an autoimmune disease is present, and the ANA test will be positive. A positive ANA will not, however, identify the specific disease. Moreover, a negative test does not rule out myositis.
Creatine Kinase (also known as CK, or Creatine Phosphokinase [CPK]) is an important diagnostic blood test for myopathies. CK is a type of protein called an enzyme that is especially active in skeletal muscle, heart tissue, and the brain. When muscle tissue is damaged, the cells release their contents into the bloodstream, causing elevated CK levels in the blood.
CK levels in the blood can vary with a number of factors, including gender, race, age, activity, health status, testing method, and more. Higher levels of serum CK can indicate muscle damage from chronic disease or acute muscle injury. In myositis, it is not uncommon for CK levels to far exceed the upper limit of normal. If the CK test indicates muscle damage, more tests will be needed to find exactly where the muscle damage occurred.
CK levels are often used to evaluate the progress of disease after treatment. This is not a reliable measure of disease activity, however, and normal enzyme levels do not completely rule out the possibility of muscle inflammation. Myositis patients sometimes wonder why they feel better or worse than their CK levels indicate. Levels may lag behind the improvement or worsening of the disease, and they may be affected by activity or other factors. Additionally, in advanced disease, serum muscle enzyme levels can be persistently low in the setting of major muscle weakness.
Sedimentation Rate (also called erythrocyte sedimentation rate [ESR] or sed rate) measures swelling and inflammation of the muscles. Doctors use the sed rate to watch the progress of the muscle inflammation. This test is not specific to a particular disease but simply identifies the presence and severity of the inflammation. It can be used to monitor the effect of treatment.
Sed rate results are reported in the distance in millimeters (mm) that red blood cells fall in a test tube in one hour (hr). The normal range varies based on laboratory equipment, age, gender, pregnancy, infection, and other factors. The sed rate will be higher with more severe inflammation.
Myositis autoantibodies are an important diagnostic and predictive tool. They are identified through a special blood test that tests for a whole panel of autoantibodies that are related to myositis diseases.
Treatment for Myositis
Myositis diseases vary widely from one patient to another, so no one treatment will work for everyone. There are a number of medications available that may be used individually or in combination to treat most forms of the disease. Sometimes, some trial and error may be needed to settle on the best treatment plan, and the risks of serious side effects must often be balanced against the therapeutic benefit of these powerful drugs. Regrettably, for some forms of myositis, particularly inclusion body myositis, there are no treatments available yet.
Because myositis and its treatments are so variable, it is also extremely important that you communicate well with your physician about your treatment, its effectiveness, and any side effects you may experience.
Keep in mind that medications are only one step in treating myositis diseases. There are many other therapies that patients find helpful and are recommended to assist you live life to the fullest, despite the challenges of chronic disease.
While polymyositis, dermatomyositis, necrotizing myopathy, and overlap disorders all, to varying degrees, are responsive to immune suppression and other therapies, sporadic inclusion body myositis still has no effective treatment. Nevertheless, research continues to better understand sIBM, and a number of compounds are currently being studied as potential treatments for sporadic inclusion body myositis.