Inflammatory myopathy

About

Inflammatory myopathy

What is inflammatory myopathy?

Inflammatory myopathy is a rare disease involving chronic inflammation and weakness of muscles with no known cause. Sometimes a rash may also develop due to this condition. It is categorized as an autoimmune disorder and there are several different inflammatory myopathy diseases present that require different treatments.

Who develops inflammatory myopathy?

This condition can affect both adults and children, so all ages are capable of developing these diseases. In certain myopathies, women are twice as likely to develop myopathy than men. All ethnic groups can develop an inflammatory myopathy. 

Where does inflammatory myopathy occur?

The main areas that inflammatory myopathy affects are the large muscles of the neck, shoulders, and hips.

What are the different types of inflammatory myopathies?

Currently, there are four chronic inflammatory myopathy conditions present. They include:

  • Polymyositis affects the skeletal muscles, therefore, impacting the movement of those diagnosed with this particular myopathy. It affects both sides of the body and is more common in women. The onset of this condition ranges from around 30-60 years of age. The prognosis for this condition varies, but most respond well with treatment therapies. If therapy is not successful, some individuals may develop severe disabilities due to this condition.
  • Dermatomyositis- includes a skin rash and progressive muscle weakness. The rash may present as purple or red spots on the upper eyelids or as scaly, red bumps over the knuckles, elbows, or knees. Women and children tend to develop this condition more. Children can develop calcium deposits underneath the skin from this condition and inflammation of the blood vessels. Lung inflammation is also common in this form, causing coughing or shortness of breath. Prognosis is good with this type of inflammatory myopathy with treatment. One-third of juvenile cases will recover.
  • Inclusion body myositis- involves progressive muscle weakness and shrinkage. People over the age of 50 and men are more susceptible to developing this type of myopathy. This type of myopathy has the poorest prognosis as it is resistant to all treatment modalities at this time.
  • Necrotizing autoimmune myopathy- symptoms of this particular myopathy are weakness in the upper and lower body, fatigue, and muscle pain. This myopathy responds the best with long-term combination immunosuppressive therapy.

Causes

What is the cause of inflammatory myopathy?

Inflammatory myopathy is an autoimmune disease where the body’s immune system attacks the healthy muscles in the body. This attack causes long-term inflammation to the muscles resulting in damage and weakness to the muscles involved.

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Symptoms

What are the symptoms of inflammatory myopathy?

Signs and symptoms of inflammatory myopathy vary but tend to involve muscles closer to the trunk of the body. The most common symptoms of inflammatory myopathy include: 

  • Fatigue
  • Loss of balance
  • Difficulty breathing
  • Cough
  • Difficulty swallowing
  • Loss of muscle strength
  • Rash
  • Minor muscle pain if any
  • Calcinosis
  • Vasculitis 

What complications are associated with inflammatory myopathy?

Adult dermatomyositis may be associated with tumors of the breast, lung, female genitalia, or bowel, so this condition should be closely monitored by healthcare providers. Some polymyositis may cause serious symptoms such as shortness of breath, heart arrhythmias, or congestive heart failure.

Diagnosis

How is inflammatory myopathy diagnosed?

Diagnosis of inflammatory myopathy will involve a complete history and physical examination. A thorough muscular exam will need to be performed. Blood tests will also be performed to look at muscle enzymes. An electromyogram (EGM) may also be considered to look for any abnormalities in the electrical activity of the muscles. A muscle biopsy may be considered to help confirm the diagnosis, and this is done by removing a small piece of muscle to be examined under the microscope. Magnetic resonance imaging (MRI) may also be used to look for any muscle abnormalities.

Treatment

What are treatment options for inflammatory myopathy?

Unfortunately, there is no cure for inflammatory myopathy. Like most autoimmune diseases, treatment is focused on managing the symptoms of the individual suffering from myopathy. Some treatment examples include:

  • Corticosteroids- usually used in polymyositis, dermatomyositis, and necrotizing autoimmune myopathy in high doses as a first line therapy to reduce inflammation.
  • Disease-modifying antirheumatic drugs (DMARDs)- immunosuppressant medications are also used for this condition to reduce inflammation but mostly when corticosteroid therapy has failed. The most common examples used for inflammatory myopathy include:
  1. methotrexate (Trexall)
  2. azathioprine  (Imuran)
  • Immunoglobulin- reserved for severe cases. This is an injectable form of medication.
  1. cyclosporine (Neoral,Sandimmune)
  2. tacrolimus (Prograf)
  3. mycophenolate mofetil (CellCept)
  4. rituximab (Rituxan)
  • Adrenocorticotropic hormone gel - injectable medication reserved when other treatment modalities have failed.
  • Physical therapy-  helpful treatment for those suffering with inflammatory myopathy to live a healthy life. It helps with treating muscle weakness and improving function.
  • Exercise- an important tool to manage myopathy complications.

Inflammatory myopathy is quite rare. However, with proper diagnosis and treatment, people can live a pain-free and normal life. There are several well-qualified healthcare providers at TeleMed2U that are capable of helping you effectively diagnose and treat this disease.

Information

Medically reviewed by:

Dr Roy Kedem, MD

Dr Zenon Andreou studied medicine at University College London, graduating in 2006. His postgraduate training was in hospitals in and around London and he trained for four years in Otolaryngology before completing his training in General practice

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