Clinical and Pathogenetic Significance of the Vasculopathy in Juvenile Dermatomyositis (JDM)
Clinical and Pathogenetic Significance of the Vasculopathy in Juvenile Dermatomyositis (JDM) is a multi-center study involving pediatric rheumatology centers in Columbus, Ohio, and Indianapolis, Indiana. Alexei Grom, MD, is the Primary Investigator of this study.
Despite the fact that juvenile dermatomyositis is viewed "primarily as a systemic vasculopathy rather than simply an inflammation of muscle and sking," our knowledge of the vascular abnormalities in this disease is based on only few descriptive histopathological studies. Such vasculopathy is often associated with the capillary necrosis that eventually leads to capillary loss and tissue ischemia, a phenomenon that is likely to be responsible for muscle infarctions and severe ulcerative complications of JDMS.
- Specific Aim #1: Will focus on detailed quantitative morphogenic characterization of JDM vascular abnormalities.
- Specific Aim #2: Will identify differentially expressed genes in muscle biopsy samples that will distinguish JDM patients with vasculopathy and tissue damage from those with relatively benign disease.
- Specific Aim #3: Will focus on gene expression profiles in peripheral blood. The long-term goal of this proposal is to understand the molecular mechanisms underlying vasculopathy and to define potential targets for therapeutic intervention.
The preliminary findings include that there is a deficient angiogenic response in muscle tissues from patients with JDM in association with over-expression of angiostatic ELR-negative CXC-Chemokines.
