PERIPHERAL MARKERS OF IMMUNE DYSREGULATION AS A MARKER OF JUVENILE DERMATOMYOSIT

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Introduction Juvenile Dermatomyositis (JDMS) is a rare, multisystem autoimmune disease that affects about 3 in 1 million children each year. Although the cause of JDMS is unknown, certain infections in genetically susceptible hosts may trigger immune mediated myositis [1-4]. Despite extensive research, the pathogenesis of Juvenile Dermatomyositis remains unknown. In addition, although treatments for JDMS have improved over the past 4 decades, patients continue to suffer considerable morbidity related to the disease process and toxicities related to medical treatments. Objectives The proposed study will examine specific peripheral lymphocyte markers in patients with JDMS. Further, we will determine whether there is a correlation between these peripheral immunologic markers and the clinical course of patients with JDMS. Aims Specific Aim 1: To conduct a prospective cohort study to describe peripheral lymphocyte markers in Juvenile Dermatomyositis (JDMS). To determine the surface markers expressed on the peripheral lymphocytes of children with JDMS. Specific Aim 2: To correlate peripheral lymphocyte markers with the clinical disease activity in JDMS patients. We hypothesize that peripheral lymphocyte markers are altered in Juvenile Dermatomyositis (JDMS) and regained at the end of disease. Based on this hypothesis we may be able to correlate peripheral immune markers with disease activity, response to therapy and remission of autoimmunity in patients with JDMS.