CMRC Children's Memorial Research Center
News 5-24-2005

 

Insights about infection and juvenile dermatomyositis
 
What problem was studied?
Juvenile dermatomyositis (JDM) causes a skin rash and muscle inflammation and weakness that can dramatically affect everyday functions such as walking and getting out of bed. In some cases, the outcome can be fatal.  While treatment options and outcomes have improved, little is known about what causes JDM. It is believed that a combination of factors, including a child’s genetic make-up and environmental triggers such as infections, might contribute to the onset of JDM. To facilitate a greater understanding of JDM, the National Institute of Arthritis and Musculoskeletal Skin Diseases (NIAMS) funded a JDM Disease Registry that has enrolled families with children with newly diagnosed JDM from all over the United States.  This study took advantage of registry families to learn more about the mix of factors that might make children more likely to develop JDM.
 
Arthritis Foundation-funded researchers involved in the study: Lauren M. Pachman, MD, Children’s Memorial Research Centter  & Rosalind Ramsey-Goldman, MD, DrPH,  both  from   Northwestern University,Feinberg School of Medicine,  Chicago, IL, supported by Arthritis Foundation Clinical Science Grants
 
What was done in the study?
The study included over 300 families of children with newly diagnosed JDM who had been enrolled in the registry. Medical records were obtained to verify the JDM diagnosis. The researchers conducted telephone interviews with the parents or other caretakers to learn about the children’s disease course and treatment, their ethnic and family background, illness history in the 3 months prior to the onset of JDM, and any prior environmental exposures such as infection, stress or toxins.
 
What were the study results?
Interviews and medical records were obtained from 286 families. JDM was twice as common in girls as in boys. The average age at disease onset was around 7 years while 25% had their first symptoms by age four or younger. In the 3 months prior to the onset of the typical JDM rash or weakness, more than half of the children had general symptoms such as fever, weight loss, fatigue or headache. Nearly 60% had respiratory symptoms such as runny nose and earache and 30% had gastrointestinal symptoms including nausea, vomiting, diarrhea or stomach pain. More than 60% of the children received antibiotic treatment for these infection-related symptoms.
 
What’s the relevance to children with arthritis?
This research shows for the first time that a significant number of the children affected by JDM are very young. The frequent occurrence of JDM following infectious symptoms provides further evidence that an infectious agent is one of the factors that leads to the onset of JDM in some children. Further study is needed to determine if these symptoms of infection are associated with a particular type of disease course and outcome. This research is important because it moves us closer to understanding the series of events at the start of the JDM’s symptoms so that we can learn how to prevent and  treat the disease early. Dr. Lauren Pachman adds, “We need families and doctors to be more aware of the JDM symptoms of rash or weakness, whether or not they  followi an infection so that children can be referred promptly to a physician who specializes in care of children with arthritis-related diseases. In related studies, we have found that early diagnosis and appropriate treatment help to improve the disease outcomes.”  
 
Source: Arthritis Care and Research/ Arthritis and Rheumatism, April  15, 2005