JUVENILE DERMATOMYOSITIS

Description:

Systemic, autoimmune inflammatory muscle disorder

Symptoms:

  • Red or purple coloring around the eyelids or cheeks
  • Muscle weakness and Muscle Pain
  • Gottron papules, heliotrope rash
  • Fatigue, Malaise, irritability
  • Sore and swollen joints
  • Frequently falling down
  • Severe abdominal pain
  • Weak voice (dysphonia)
  • Difficulty swallowing (dysphagia)
  • Deposits of calcium underneath the skin (calcinosis)
  • Contractures, Ulcers

Causes:

  • autoimmunity
  • Genetic predisposition
  • abnormal immune system response to a virus
  • Infection

Risk Factors:

  • Chronic disease
  • 3000 to 5000 children affected in the U.S per year
  • Girls are twice at risk to be affected as boys
  • Children at any age can be affected
  • Risk of developing Diabetes, celiac disease and arthritis

Diagnostic Tests:

  • Blood tests(used to monitor enzymes or antibodies)
  • Magnetic resonance imaging
  • Electromyography
  • Muscle biopsy
  • Nailfold capillaroscopy
  • Skin biopsy,
  • lung function tests, ECG (heart function), abdominal ultrasound,
  • CT scan and EMG (muscle test)

Differential Diagnosis:

  • Swallowing-Speech Language therapy assesment & video fluroscopy
  • Lungs-Chest Xray
  • Typical rash, typical pattern of muscle weakness
  • Muscle enzyme elevation in the blood tests
  • Tests of muscle strength

Treatments:

  • Physical and occupational therapy
  • Skin protection
  • Diet assessment
  • alternative therapies, such as reiki, acupuncture and massage

Medications:

  • Corticosteroids (to slow down body’s autoimmune response)
  • Use of Disease modifying anti-rheumatic drugs (DMARDS)
  • Immunosuppressants (quiet the immune system so it does’nt overreact)
  • Intravenous immune globulin (helps quiet the immune response)
  • hydroxychloroquine
  • Bisphosphonates
  • Drugs to reduce inflammation
  • Supplements such as Folic acid, Calcium and Vitamin D

Lifestyle Management:

  • adequate and balanced diet
  • Photo-protective clothing
  • Exercise(to improve muscle strength and endurance)
  • Sport should be limited (to avoid injuries)
  • Use of sunscreens
  • Maintaining good general health