Cytokines in idiopathic inflammatory myopathies

Recent findings suggest cytokines as important key molecules in the pathogenic mechanisms of idiopathic inflammatory myopathies, myositis. In this review, we focus on cytokines with a potential role in disease mechanisms in myositis and present some general information on individual cytokines and an updated summary from the literature concerning cytokines in these disorders. The idiopathic inflammatory myopathies is a heterogeneous group of disorders clinically characterized by symmetric proximal muscle weakness and by certain defined histolopathological findings, including inflammatory infiltrates in muscle tissue. Other prominent findings in the target tissue of these patients are defined molecular changes of blood vessels and muscle fibers, including reformation to high endothelial venule (HEV)-like blood vessels and intensive MHC class I expression in muscle fibers. The predominant clinical symptoms of muscle weakness and decreased muscle endurance are shared by all subsets of inflammatory myopathies and indicate that some pathogenic mechanisms related to muscle function may be shared by the different disease groups. Studies on cytokine gene, RNA and protein expression in muscle tissue from patients with various forms of the disease also indicate similar profiles, despite different phenotypes of the inflammatory cells present in muscle tissue from the different subsets of myositis. There is a pronounced expression of various cytokines in muscle tissue, among which the proinflammatory cytokines TNF-alpha and IL-1 are most widely explored in the inflammatory myopathies, which has made them into potential therapeutic targets. The use of targeted cytokine therapy has been successful in several other chronic inflammatory diseases and although the exact role of cytokines in chronic idiopathic inflammatory myopathies remains to be delineated their potential role as targets for new therapies in this disorder will be discussed in this review.