Novel Therapeutic Options in Treatment of Idiopathic Inflammatory Myopathies

The purpose of this review is to update the audience on the recent progress in the treatment of idiopathic inflammatory myopathies, highlighting a myriad of treatment trials aimed at slowing down progression of muscle weakness and/or skin involvement in idiopathic inflammatory myopathies.

Myositis continues to be an active area of clinical and translational research. Through the work of a number of investigators, we now have a much better understanding of the pathogenesis underlying the various myositides. The role of T cells, B cells, and dendritic cells has been better defined in dermatomyositis and inclusion body myositis. The role of autoantibodies has been better elucidated, with the recognition that autoantibodies play an important role in not only establishing the diagnosis but also helping with prognostication and choosing treatment paradigms. A number of new treatment trials were designed to alter the pathogenic factors, including T cell activation, docking, and signaling, cytokines, B cell signaling, B cell depletion, as well as targeting Toll-like receptors (TLRs). Myostatin inhibitors have been developed and tried in inclusion body myositis (IBM) in hopes that they would stop muscle atrophy and improve muscle strength and function. This review will outline the progress made to date in the treatment of idiopathic inflammatory myopathies.