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Juvenile Myositis

Juvenile Myositis (JM) is found in children under the age of 18 and affects 3,000 to 5,000 children in the United States. The most frequent form of JM is juvenile dermatomyositis (JDM), in which children experience marked muscle weakness and skin rash. The other form of myositis that can occur in children—juvenile polymyositis—is extremely rare.

The first sign of JDM is usually a skin rash. The rash may be red and patchy, like dry skin; a red or purplish color on the eyelids or cheeks that may look more like allergies; or both. Children with juvenile polymyositis do not experience skin symptoms.

JDM patients can have weak muscles at the same time they see the skin rash, or the weakening muscles may occur after the rash over days, weeks, or months. The weaker muscles are usually those closer to the body, in the neck, shoulders, back, and torso. The child may have trouble climbing or standing from a seated position. The skin rash and weak muscles are caused by inflammation or swelling in the blood vessels under the skin and in the muscles.

Risk Group

Children from the ages of 5-15 are the primary patients of JM. JM affects 3-5,000 children in the United States.

Symptoms

  • Reddish-purple rash over the eyelids or joints

  • General tiredness

  • Moodiness or irritability

  • Complaints of tummy aches

  • Trouble climbing stairs or onto a bus, standing from a seated position, or getting dressed

  • Difficulty reaching up, like to shampoo or comb hair

  • Trouble lifting the head

  • Swelling or redness in the skin around the fingernails

  • Characteristic rashes of JDM—Gottron’s papules (rashes or lesions over the knuckles, elbows, and knees) and heliotrope rash (purplish rash around the eyes)

  • Gradual muscle weakness, most often those closest to the body such as neck, stomach, upper arms, and legs

  • Hardened lumps or sheets of calcium (calcinosis) under the skin

  • Trouble swallowing (dysphagia)

  • Hoarse-sounding voice

Diagnostic Tests

  • Your doctor will ask for a complete medical history and will perform a thorough physical examination.

  • Certain blood tests will be ordered and a muscle biopsy may be performed. The muscle biopsy is a minor procedure. A local anesthetic is applied and a small piece of muscle is removed -usually from a thigh muscle or shoulder muscle.

Treatment

Your doctor will most likely prescribe medications including corticosteroids and immunosupressants and will suggest certain physical exercises to help improve and maintain muscle strength.

Exercise and physical therapy are important parts of standard myositis treatment plans. Physical exercise has been shown to reduce inflammation, reduce fatigue, increase stamina, and build muscle, even in patients with myositis. Indeed, exercise is currently the only treatment recommendation for patients with inclusion body myositis.

There is a strong association between aerobic capacity and general health, both in healthy individuals and those with myositis. Regular physical activity and exercise can improve one’s quality of life and reduce the risk of serious chronic diseases, such as type II diabetes, osteoporosis, hypertension, and cardiovascular disease. These are all complications of myositis diseases or their treatment, so exercise is doubly important.

 

 

Copyright © 2018 Johns Hopkins Myositis Center

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