The goal of treatments for juvenile dermatomyositis is to minimize inflammation, improve function, and prevent disability. The treatment should be early and requires a team approach between the rheumatologist, physical therapist, dermatologist, and primary care doctor. JDM is unique among most rheumatic diseases of childhood. Many children have symptoms that do go away with treatment; however, most are not cured.
Corticosteroids
Corticosteroids alter the immune system, limiting the production of antibodies and reducing skin and muscle inflammation, as well as improving muscle strength and function. Corticosteroids, especially prednisone, are usually the first choice in treating inflammatory myopathies such as dermatomyositis, because they work quickly.
The doctor may start with a very high dose, often intravenously (administered directly into vein), and then decrease it as signs and symptoms improve. Signs of improvement may be seen in about two to four weeks as the inflammation decreases, but full recovery will not be seen for months after initiating treatment. Often, physical therapy is required for strengthening and retraining the muscles that were damaged.
Standard treatment for JDM has been high-dose daily oral glucocorticoids (e.g., up to 2 mg/kg/day of prednisone, at times in divided doses), which is continued until clinical and laboratory improvements are evident and then reduced slowly. Intravenous glucocorticoids (methylprednisolone) are also often used at the beginning of therapy. Most patients develop treatment-related side effects with the use of steroids. In many cases, however, prednisone is introduced early as a treatment option and may be discontinued before the two-year period is completed.
Prolonged use of corticosteroids can have serious and wide-ranging side effects, like weight gain, osteoporosis, and cataracts, so the doctor may recommend supplements like calcium and vitamin D to strengthen bone and regular eye exams to detect cataracts.
Corticosteroid-sparing agents
Other medications work more slowly, but have fewer side effects than corticosteroids like prednisone, and allow the patient to wean off steroids sooner (“spare” the steroids).
Other aspects of treating juvenile dermatomyositis include:
- Skin protection
Protection from ultraviolet A and B (UVA and UVB) light is thought to help control the rash and potentially prevent muscle disease. Use sunscreen or sunblock that decreases exposure to UVA and UVB light. Wear wide-brimmed hats and photo-protective clothing. Avoid sun exposure during peak daylight hours.
- Physical therapy
A physical therapist can teach exercises to maintain and improve strength and flexibility and advise an appropriate level of activity. Physical activity is thought to be important in JDM. Physical therapy is directed at preventing muscle wasting and stiffness, and is particularly necessary in patients with calcium deposits (calcinosis) and muscle involvement. Therapy should focus initially on stretching and splinting and only include more aggressive strength-building therapy once inflammation is controlled.
- Speech therapy
If the swallowing muscles are weakened by dermatomyositis, speech therapy can help the patient learn how to compensate for those changes.
- Diet assessment
In JDM, chewing and swallowing can become more difficult. A registered dietitian can teach how to prepare foods that are safe to eat.