Juvenile dermatomyositis (JDM) is a connective tissue disease that causes skin rash and weak
muscles in children. The purpose of this study is to measure the absorption of oral
prednisolone and intravenous (IV) methylprednisolone and to determine levels of disease
activity indicators in the blood. These levels will be compared to see if there are patterns
specific to active and less active JDM.
JDM is a connective tissue disease that is characterized by inflammation of the muscles and
the skin. Corticosteroids, such as prednisolone and methylprednisolone, can be administered
to help control symptoms of the disease, but absorption patterns of these medications in
oral and IV forms are unknown. This study will assess absorption of oral prednisolone and IV
methylprednisolone, measure levels of two disease activity indicators (von Willebrand factor
and neopterin), and correlate these values in children with JDM.
Patients will participate in this study twice within a period of up to a year, once when the
patient's disease is active, and again 6 to 12 months later when the disease is less active.
Each of the two study periods will last two nights and two days. Patients will be admitted
to the hospital the first night, and a small IV port will be inserted in the patient's arm
the first morning to allow for multiple blood draws without additional needle sticks.
Patients will receive oral prednisolone the first morning and IV methylprednisolone the
second morning. Baseline blood draws will be performed prior to administration of drug, with
13 additional draws over a 6 hour period following drug administration. Following the final
blood draw on the second day, the IV port will be removed from the patient's arm and the
patient will be discharged from the hospital.
Blood drawn from patients will be assessed for absorption of the drugs and levels of von
Willebrand factor and neopterin. Patients will undergo the same sequence of events sometime
between 6 to 12 months after the first hospitalization, after their vasculitis is judged to
be less active.
- Juvenile dermatomyositis with evidence of active vasculitis
- Elevated von Willebrand factor antigen prior to study entry
- Elevated neopterin level prior to study entry
- Severe renal involvement
- Critically ill or clinically unstable
- Diseases other than dermatomyositis with vasculitis