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Polymyositis - adult

Definition

Polymyositis is a rare inflammatory disease. It leads to muscle weakness, swelling, tenderness, and tissue damage. It is part of a larger group of diseases called myositis.

Causes

Polymyositis affects the skeletal muscles. It is also known as idiopathic inflammatory myopathy. The exact cause is unknown, but it may be related to an autoimmune reaction or infection.

Polymyositis can affect people at any age. It is most common in adults between ages 50 and 60, and in older children. It affects women twice as often as men. It is more common in African Americans than Caucasians.

Symptoms

Polymyositis is a systemic disease. This means it affects the whole body. Muscle weakness and tenderness can be signs of polymyositis. A rash is a sign of a similar condition, dermatomyositis.

Common symptoms include:

  • Muscle weakness in the shoulders and hips. This can make it hard to raise the arms over the head, get up from a sitting position, or climb stairs.
  • Difficulty swallowing.
  • Muscle pain.
  • Problems with the voice (caused by weak throat muscles).
  • Shortness of breath.

You may also have:

  • Fatigue
  • Fever
  • Joint pain
  • Loss of appetite
  • Morning stiffness
  • Weight loss

Exams and Tests

Tests may include:

People with this condition also must be watched carefully for signs of cancer.

Treatment

The main treatment is the use of corticosteroid medicines. The dose of medicine is slowly tapered off as muscle strength improves. This takes about 4 to 6 weeks. You will stay on a low dose of a corticosteroid medicine after that. Medicines to suppress the immune system may be used to replace the corticosteroids. These drugs may include azathioprine, methotrexate or mycophenolate.

For disease that remains active in spite of corticosteroids, intravenous gamma globulin has been tried with mixed results. Biologic drugs also may be used. Rituximab appears to be the most promising. It is important to rule out other conditions in people who do not respond to treatment. A repeat muscle biopsy may be needed to make this diagnosis.

If the condition is associated with a tumor, it may improve if the tumor is removed.

Outlook (Prognosis)

Response to treatment varies, based on the complications. As many as 1 in 5 people may die within 5 years of developing the condition.

Many people, especially children, recover from the illness and do not need ongoing treatment. For most adults, however, immunosuppressant drugs are needed to control the disease.

In adults, death may result from:

  • Malnutrition
  • Pneumonia
  • Respiratory failure
  • Severe, long-term muscle weakness

The major causes of death are cancer and lung disease.

Possible Complications

Complications may include:

  • Calcium deposits in the affected muscles, especially in children with the disease
  • Cancer
  • Heart disease, lung disease, or abdominal complications

When to Contact a Medical Professional

Call your health care provider if you have symptoms of this disorder. Seek emergency treatment if you have shortness of breath and difficulty swallowing.

References

Amato AA. Disorders of skeletal muscle. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 110.

Greenberg SA. Inflammatory myopathies. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 269.

Nagaraju K, Lundberg IE. Inflammatory diseases of muscle and other myopathies. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley and Firestein's Textbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017:chap 85.

Oddis CV, Reed AM, Aggarwal R, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum. 2013;65(2):314-324. PMID: 23124935 www.ncbi.nlm.nih.gov/pubmed/23124935.

Review Date:2/8/2017
Reviewed By:Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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Outcome Data

No data available for this condition/procedure.

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