Immunomodulatory treatment for dermatomyositis.

Dermatomyositis and polymyositis are classified as members of idiopathic inflammatory myopathies. It has recently become evident that the pathogenesis of the muscle disease for these two entities is likely not the same. Despite this observation, therapies directed at the muscle disease are similar, although therapy for the skin differs slightly. Corticosteroids remain the initial approach to management. The systemic dose of corticosteroids needed to control the disease, particularly the skin disease, is quite high; therefore, most authorities add a corticosteroid-sparing agent early. Some patients will naturally "burn out" the inflammatory component of their disease, but many have continuing disease activity that requires maintenance therapy. In this article, we briefly define the disease state of dermatomyositis, discuss the associated systemic manifestations, and update the selection and evaluation of immunomodulatory, corticosteroid-sparing agents.