Pathogenesis and therapy of inclusion body myositis

Abstract

Purpose of review: Inclusion body myositis (IBM) is a poorly understood progressive muscle disease of middle and later life. Its dual pathologies of autoimmunity and unexplained myofiber degeneration and loss have been enigmatic since its earliest descriptions over 40 years ago. No reliable effective therapy currently exists for IBM. This review provides an update of current issues in the pathogenesis and therapy of IBM.

Recent findings: Recent studies have further defined the clinical features of IBM, including natural history, pattern of muscle involvement, and role of MRI imaging. Further potential immune mediators have been identified. An autoantibody directed against a muscle antigen appears to have high specificity for IBM among muscle diseases. Further evidence for myonuclear degeneration has been reported.

Summary: IBM remains a poorly understood muscle disease, although understanding of the pathophysiological mechanisms continues to expand and is supporting new therapeutic approaches.