What is Myositis? Myo (muscle) Itis (Inflammation) means Myositis is the inflammation of muscles. Myositis is a rare autoimmune disease with variations in manifestation. As with all autoimmune diseases the immune system becomes confused and believes healthy and normal tissues of the body are an enemy, with Myositis the immune system believes muscles are the enemy and causes the immune system to attack and inflame the muscles of the body. In the United States, there are an estimated 1,600 to 3,200 new cases per year and 50,000 to 75,000 people living with myositis. (Thornton, 2019) Myositis can affect anyone at any age, including children. The main muscles that are generally inflamed predominantly are hips, shoulders, thighs, and trunk muscles. Two most common variations of Myositis are Dermatomyositis and Polymyositis, the article today will have in depth details for both. Healthy lifestyles are imperative for all rheumatic diseases.
Dermatomyositis is not only exclusively rare but also can be complex to pronounce. Dermatomyositis (dur-muh-toe-my-uh-SY-tis) is a rare inflammatory disease marked by muscle weakness and a distinctive skin rash. The rash associated with DM generally makes its’s appearance prior to the onset of muscle inflammation and weakness. Rash is referred to as “butterfly’ rash it appears on upper eyelids and across the cheeks, the rash is purple in color that migrates to red in tone. Muscle weakness is next to begin after onset of the initial rash and weakness begins in trunk muscles, thighs, neck, and abdominal. In children with dermatomyositis, calcium deposits can occur at the sites of inflammation, and may further restrict movement. The muscle weakness tends gradually get worse if not caught early and treatment provided quickly.
Dermatomyositis remains an incredibly rare autoimmune disease the diagnostic tools and procedures have improved over the last few years. First and foremost, during a suspected autoimmune or disease manifestation is an evaluation appointment with a licensed Rheumatologist. Rheumatologists are specialists of rheumatic diseases and best fit to serve the autoimmune and DM population as they best recognize onset and treatment.
During the evaluation appointment questions regarding onset, symptoms, and daily living problems will be asked. Since DM presents in muscles questions such as “is it difficult for you to get in and out of bed or chair and has this difficulty always been there or new?” can be asked to establish baselines of when muscle wasting may have begun. Physical exams will be important to understand muscle strength and review of the skin to observe any rashes.
The most common diagnosis tool for Dermatomyositis is a blood panel and second to that a muscle biopsy. The serum activity level of creatine kinase (CK) is the most performed enzyme test for the diagnosis and monitoring of myositis. (Volochayev et al., 2012) Additionally, myositis antibodies can be screen for as well. Muscle biopsies can be taken to also screen for Myositis.
Overall objective for all autoimmune and rheumatic disease treatment is settle the hyperactive immune system. The immune system becomes so agitated and confused that it attacks the body itself. Treatment has the goal of getting the immune system to stabilize. Corticosteroids and immunosuppressants are the most common medicinal treatments such as Methotrexate and Prednisone. Physical therapy and exercise are important for patients once bloodwork stabilizes with ongoing inflammation. Myositis patients can continue to pursue movement to achieve strength of muscles and in some cases build back muscle lost during initial onset of the disease. Incorporating a healthy diet filled with anti-inflammatory foods can also assist in inflammation reduction and muscle performance. Patients with Myositis may want to turn away from common foods that can increase inflammation such as friend foods, oils, and sugars.
Lifestyle after Diagnosis
Completion of diagnosis and control of the inflammation and heightened immune response, it is important for DM patients to stay active and participate in a healthy diet. Mobility is crucial for continued us of muscles and for wasting to not occur, and healthy food choices to partner with the medications in reducing inflammation. Sun exposure should be limited while taking steroids or immunosuppressants and regular testing should occur as ordered by the patient’s rheumatologist.
For a quick guide on supplements that can assist with inflammation check out our post: here
Polymyositis manifests in similar fashions as Dermatomyositis, however, it does not have skin involvement and creates ongoing muscle weakness in a slightly slower fashion than DM. (PAH-lee-MY-oh-SY-tis) An inflammatory disease of the muscles closest to the center of the body. It causes weakness, inability to stand, climb stairs, lift, or reach. It may also cause muscle pain and difficulty swallowing. There are also juvenile manifestations of Polymyositis but rare. Polymyositis occurs most often in adults and older adults. In the US, there are less than 5,000 with this disease. (Polymyositis – about the disease 2021) An onset cough and joint spasms can occur during a flare of Polymyositis.
PM and DM are idiopathic inflammatory myopathies, characterized by the shared features of proximal skeletal muscle weakness and evidence of muscle inflammation. Differences between two in beginning manifestation may be slight. As discussed, DM typically first presents with rash, and then later if not diagnosed and treatments beginning muscle inflammation and possible muscle wasting. However, with PM no rash and muscle inflammation and wasting occurs at a steady rate until immune suppression and control occurs.
Polymyositis patients are most commonly under the care of Rheumatologist as their specialty and expertise proves most beneficial for the management of the disease. Physical evaluation and history should be taken during an initial appointment to understand if the paid and muscle inflammation is new, an immune response, and rule out other diseases or injury causing the initial complaint. Joint tests will be performed to screen for Rheumatoid Arthritis as well as muscle tests during evaluation exam such as raising arms, lifting legs, and laying down and sitting back up. These tests let the physician know if the muscle inflammation has begun to create muscle wasting or destruction of muscles that are under attack.
Official diagnosis begins with blood panels. CK, ESR, and Aldolase are often screened to review the level of inflammation in the body and better assists the specialist on the amount of treatment to begin with. Additionally, imaging and biopsies can also be used to formally diagnose PM. The typical MRI ordered is a bilateral thigh MRI using a myositis protocol. Despite being nonspecific for myositis, MRI can be particularly useful in helping to determine whether muscle disease is active in a patient with known DM or PM, especially in situations in which the patient’s serum muscle enzymes are not reflective of the patient’s muscle disease activity. (Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults 2020)
Polymyositis is a very treatable disease and some people may recover completely. In order to gain immediate control over the immune system and the attack it places on the body during a PM flare a high dose of Corticosteroids may be required initially. Prolonged use of steroids can have long term negative effects on the body so a physician will attempt to taper off steroids as quickly as possible. The two other most common medications used for Polymyositis are azathioprine and methotrexate both of which are immunosuppressants to gain control over the immune system and keep it from causing inflammation and attacking the body and muscle tissue.
Polymyositis will require physical therapy if the muscle wasting caused too much muscle weakness for daily living requirements to occur such as going to the bathroom, dressing, showering, and eating. Swallowing and facial muscles may be affected by PM causing an inability to swallow or speak. It is possible that speech and swallowing therapies may be needed dependent on degree of manifestation.
Some people may need a cane, walker or even a wheelchair for flare-ups of PM. Plasmapheresis, a “blood-cleansing” process to remove antibodies, was at one time used in PM but is rarely used today. Immunosuppressant drugs and/or IVIg treatments are now considered more effective.
Lifestyle after Diagnosis
While a patient is experiencing their PM flare it is best to take medications and therapies as prescribed and best attempt to reduce lifestyle stress. During a flare a patient may need to take some time off of work in order to fully recuperate. Rest and stress free environments are the healthiest for a PM patient to reach remission of their disease. Once the ESR and CK bloodwork levels begin to reduce demonstrating reduction of inflammation the physician may begin to reduce pharmaceutical drugs. Drinking lots of water and as remaining as mobile as possible will be critical for rebuilding muscle lost and keeping joints lubricated for pain reduction.
Once remission is reached importance on physical exercise and healthy diet should be stressed. In addition to pharmaceutical drugs there are healthy supplements and diets that can support the immune system and naturally reduce inflammation in the body. Our article here shares some of those tips: click here
A healthy lifestyle high in omega 3 fatty acids can greatly benefit keeping PM in remission and reduce likelihood of flares in the future.
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Thornton, C. (2019, February 8). Celebrate your rare. The Myositis Association. Retrieved January 31, 2023, from https://www.myositis.org/blog/celebrate-rare/#:~:text=Myositis%20is%20a%20rare%20muscle,million%20people%20in%20this%20country.
Volochayev, R., Csako, G., Wesley, R., Rider, L. G., & Miller, F. W. (2012). Laboratory test abnormalities are common in polymyositis and dermatomyositis and differ among clinical and demographic groups. The open rheumatology journal. Retrieved January 31, 2023, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3377888/#:~:text=The%20serum%20activity%20level%20of,1%2C%204%2C%205%5D.
U.S. Department of Health and Human Services. (2021, November 8). Polymyositis – about the disease. Genetic and Rare Diseases Information Center. Retrieved January 31, 2023, from https://rarediseases.info.nih.gov/diseases/7425/polymyositis
Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults. UpToDate. (2020, December). Retrieved January 31, 2023, from https://www.uptodate.com/contents/diagnosis-and-differential-diagnosis-of-dermatomyositis-and-polymyositis-in-adults