Dermatomyositis

We have several projects focused on understanding the mechanism of disease for dermatomyositis and developing therapy for it. Our dermatomyositis research has identified the presence in dermatomyositis muscle of immune cells specializing in the production of interferon-α. These cells are called plasmacytoid dendritic cells. Genes induced by interferon-α are highly active in dermatomyositis muscle. We believe that overactivation of this pathway is central to the injury to capillaries and muscle in this disease.

Our specific research projects in this area are:

• Interferon–α/β–mediated innate immune mechanisms in dermatomyositis. This project has looked at the pattern of gene expression present in dermatomyositis muscle tissue and found marked and specific overexpression of an innate immune system pathway in that disease. Immune cells that produce interferon-α were identified in DM muscle tissue, and the characteristic pathological lesions of dermatomyositis – perifascicular atrophy and capillary injury – were furthermore linked to overactivation of this pathway. Details pertaining to the publication of these results are found here. Subsequently, related findings have been observed by others in the skin lesions of dermatomyositis (see Wenzel et al. 2006)
• Blood gene expression profiles in dermatomyositis
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• Addressing uncertainties in models of dermatomyositis
• Drug development for dermatomyositis. This project’s results have not been published. The description will be updated when results are published.