This finding is important because a central question in JDM research has always been: Do the dendritic cells get activated in muscle tissue? Or, do they get activated outside of the tissue? The research by Mayo Clinic and collaborators provides the first proof that the dendritic cells get activated inside muscle tissues and then may move out into the bloodstream. Says Dr. Reed: "When you think about it as a clinician, it's really exciting because it shows what is happening in the muscle that starts the disease -- and holds out the possibility that it is maybe something that we can turn off in new treatments we develop by targeting the mechanism in the muscle tissue. And that's really neat stuff for our patients."
JDM is a rare (5 in 1 million children) autoimmune disorder of young children characterized by inflammation of the blood vessels under the muscle and skin. This results in muscle damage, as well as in tissue changes of skin over the eyelids, finger joints and knuckles. Symptoms appear gradually and include: muscle pain and tenderness; difficulty swallowing, which results in weight loss; irritability; fatigue; fever; and rash around the eyelids, finger joints, knuckles, elbows, ankles or knees.
Diagnosis may involve the following: blood tests to detect muscle enzymes and markers of inflammation; an electromyography (EMG) to assess nerve or muscle damage; muscle biopsy for examination; X-rays; and MRI. While there is no cure for JDM, there are treatment options. They include medications to reduce inflammation and skin rashes; physical and occupational therapy to improve
Contact: Lisa Lucier