There is no cure for JDM. However, there are treatments that can reduce or eliminate the symptoms. This allows people with JDM to lead active, healthy lives.
The goals of any treatment program for JDM are to control muscle inflammation and damage, maintain and improve muscle strength and function. Other goals are to get rid of pain, control or prevent other symptoms. Also, a goal is to help the child and the family learn to live with the illness.
To reach these goals, doctors work with families to find a treatment plan that works best for them. Treatment includes medication, physical therapy, and education. As your child's symptoms change, the treatment plan may also be changed. Some common drug therapies include:
Prednisone is the most commonly used drug in the group of medications called steroids, corticosteroids, or glucocorticoids. Other drugs in this group are Solumedrol or Prednisolone. Prednisone (or one of the other steroids) is the first treatment for children with JDM in most cases. Prednisone works quickly to control inflammation in the muscles, joints, and skin.
This drug is similar to cortisone, a hormone produced naturally in the body. At first, high doses of this drug are given until your child's muscle enzyme tests and strength improve. As your child gets better, the dose will be slowly reduced. In most cases children will remain on prednisone for at least two years.
Side effects of prednisone depend on both the dose used and the length of therapy. Common side effects include weight gain, increased appetite, mood changes, increased risk for infections and facial swelling. Prednisone suppresses the immune system. This is important in suppressing the inflammation. But it also increases the risk for infection. The amount of immune suppression is related to the dose. Over a long period of time, the drug can also cause decreased calcium content in the bones, cataracts, high blood pressure, and slowed growth rate. The doctor will lower the dose as soon as possible and may also start other medications to help control the JDM so to decrease the prednisone side effects, while keeping the disease under control.
For almost all patients with JDM, methotrexate is started at the same time or very soon after prednisone. Methotrexate is used to help control JDM. It also decreases the amount of steroids needed. Methotrexate suppresses the immune system. Methotrexate is also used to treat cancer but in much higher doses. Methotrexate has been used in the treatment of children with JDM for over 30 years.
The side effects from methotrexate in children with JDM are less common and milder than when it used to treat children with cancer. It will be used with prednisone. Methotrexate can be given as a pill or by injection just under the skin like an insulin or allergy shot. It is given once a week.
Common side effects include nausea, feeling tired for about 12-24 hours after the weekly dose and mouth sores. Folic acid is a vitamin that can make the side effects better. Less common side effects are increased liver enzymes and an increased risk of getting infections.
Hydroxychloroquine (brand name Plaquenil)
Hydroxychloroquine is a drug that is used to treat the rash in children with JDM. The drug is given as a pill. It is given once a day. It is given along with other drugs that control the other symptoms in children with JDM.
Hydroxychloroquine is generally tolerated very well. Some children will have stomach upset. About one in every 3,000-5,000 people who take hydroxychloroquine will gather pigment in the retina of the eye. If this continues, it could interfere with a person's vision. For this reason, an eye doctor needs to check your child's eyes every 12 months while your child is taking hydroxychloroquine. If the eye doctor finds any pigment accumulation, the hydroxychloroquine will be stopped before any visual problems develop.
Cyclosporine (brand names Neoral or Sandimmune)
If prednisone and methotrexate don't fully control your child's JDM, your doctor may use cyclosporine. This is another type of drug that decreases inflammation by suppressing the abnormal immune response in children with JDM It was originally developed for organ transplants. Cyclosporine, along with methotrexate, can help better control the inflammation. This means that lower prednisone doses can be used with less risk for repeat episodes of disease.
Side effects that may occur include extra hair growth on the face and arms, swelling of gums, and high blood pressure. It can also injure the kidney.
Intravenous Immunoglobulin (IVIG)
IVIG is a blood-derived product that has been used in the treatment of children with JDM. IVIG consists of purified antibodies. Antibodies are proteins that the body's immune system uses to fight infection. IVIG has been shown to slow down the inflammatory process. IVIG is given through a needle into a vein (intravenous or IV) in the hospital once a month.
Side effects like headache, fever, and vomiting can occur. They can be prevented by pretreating with Tylenol, Benadryl, and sometimes the addition of a steroid.
Mycophenolate Mofetil (Cellcept)
Cellcept is a medication that is becoming more often used to treat severe disease if it is not well controlled by the medications listed above. Cellcept is a powerful immune suppressive drug that is used to treat severe lupus and other autoimmune diseases. It comes as a capsule, pill, or liquid and is taken twice a day.
Side effects include nausea, abdominal pain and increased risk of infection. While taking this medication, your child’s doctor will regularly monitor blood cell counts. This is done to make sure the risk of infection does not become too great. This medicine can be very helpful in lowering prednisone doses.
Sun exposure can make the rash worse or develop in new areas for many children with JDM. Severe sun exposure can make the overall disease worse. It is important that a sunscreen made with zinc and titanium of at least SPF 30 is used every day the child is outside, including winter. It should be reapplied every 2 hours to all areas of the skin that are not covered by clothing.
The treatments described here are the most commonly used. In addition, new treatments are being tested in the treatment of JDM. You should discuss all treatment options with your child’s doctor.