What Is Juvenile Dermatomyositis & How Is It Treated?|Causes, Symptoms, Prognosis of Juvenile Dermatomyositis

What is Juvenile Dermatomyositis?

Juvenile dermatomyositis (JDM) is an autoimmune disease in children, which causes skin rash, tissue damage and muscle inflammation (myositis), resulting in weakening of muscles. Juvenile dermatomyositis differs from adult dermatomyositis and is not associated with increased risk of cancer. However, Juvenile dermatomyositis (JDM) often involves severe blood vessel involvement, and calcinosis also occurs in this disease, especially in its recovery phase. Juvenile dermatomyositis (JDM) occurs throughout the world but more frequently in North America, especially in the African-American population. Juvenile dermatomyositis mainly affects children aged less than 16 years. Even though Juvenile dermatomyositis (JDM) develops before the age of 16 years, it can persist into adulthood in some people. The average age of onset for Juvenile dermatomyositis or JDM is 7 years and it rarely occurs in infants aged less than 2 years. JDM affects girls twice as more as boys.

What is Juvenile Dermatomyositis?

What Causes Juvenile Dermatomyositis?

The causes of Juvenile dermatomyositis (JDM)are still unknown. However, researchers suspect myositis to be triggered by the combination of two factors, infection and genetics. According to this theory, the body responds abnormally to a virus infection. The immune system attacks and eliminates the virus, but does not stop this process and starts attacking the body’s own tissues. Although not a simple genetic inheritance like other muscle disease, juvenile dermatomyositis, however, is a partially inherited disease. Juvenile dermatomyositis (JDM) is not a contagious disease.

What are the Symptoms of Juvenile Dermatomyositis (JDM)?

The symptoms of juvenile dermatomyositis can range from mild to severe. However, these are rarely life-threatening. At its onset, Juvenile dermatomyositis (JDM) is primarily characterized by symptoms like fever ranging from 101-104º, skin rash, muscle weakness, stiff and swollen joints, contractures, ulcers, calcium deposits in the body, redness and dryness of skin, poor appetite, weight loss and gastrointestinal problems. Children with juvenile dermatomyositis get fatigued easily, lack energy, perform fewer activities or refuse to do certain tasks. There are certain times when children with Juvenile dermatomyositis (JDM) do not have any symptoms. This is known as the remission.

How is Juvenile Dermatomyositis (JDM) Diagnosed?

There are no specific blood tests to detect Juvenile dermatomyositis (JDM). To diagnose juvenile dermatomyositis, the doctor would initially ask the parents about the child’s symptoms, and then examine the child physically to check for rashes involving the face, eyelids, knuckles, elbows and knees. The doctor would also conduct a muscle strength exam and muscle biopsy, if required. They may order for MRI scans to spot the inflamed muscles, and also blood and urine test to detect any increase in the level of one or more muscle related enzymes like aldolase, CPK and SGOT.

How is Juvenile Dermatomyositis Treated?

There is no cure for juvenile dermatomyositis. However, there are effective treatments, which can reduce or eliminate the symptoms and help Juvenile dermatomyositis (JDM) patients lead an active and healthy life. All treatment programs for Juvenile dermatomyositis (JDM) aim to control muscle damage and inflammation, relieve pain, improve and maintain muscle strength and function, and manage or prevent other symptoms. These treatments help the child and their family to manage juvenile dermatomyositis more efficiently and effectively.

To attain these goals, doctors work with the families of juvenile dermatomyositis patients to find a treatment plan which best suits the patient. Treatment for juvenile dermatomyositis generally involves physical therapy, medication and education. As the child’s symptoms change, the treatment plans are also altered accordingly. Some common drug therapies for Juvenile dermatomyositis include prednisone, methotrexate, hydroxychloroquine, cyclosporine, mycophenolate mofetil and intravenous immunoglobulin.

What is the Prognosis for Juvenile Dermatomyositis (JDM)?

Juvenile Dermatomyositis is a treatable health issue. Most children enter remission within 2 years and can have their medications eliminated. However, in certain cases juvenile dermatomyositis remains active for longer than 2 years, and some patients have more severe symptoms than others. Some children with Juvenile dermatomyositis (JDM) experience repeated episodes of the disease or may have the kind of disease which does not respond easily to medications. It is not possible to predict how a child will respond to medications and treatments. So, it is all the more important that Juvenile dermatomyositis patients regularly take all the medicines prescribed by their doctor and perform physical therapy. Regardless of the challenges faced by children with Juvenile dermatomyositis (JDM) and their families, majority of these kids grow up to lead a productive and active life.

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