title: Pathogenesis and therapy of inclusion body myositis.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  IBM remains a poorly understood muscle disease, although understanding of the pathophysiological mechanisms continues to expand and is supporting new therapeutic approaches.
authors.affiliation: Division of Neuromuscular Disease, Department of Neurology, Brigham and Women's Hospital, and Children's Hospital Informatics Program Harvard Medical School, Boston, Massachusetts 02115, USA. sagreenberg@partners.org
authors.forename: Steven A
authors.initials: SA
authors.lastname: Greenberg
mesh_terms: D000818:Animals; D015551:Autoimmunity; D002467:Cell Nucleus / Q000473:pathology; D006801:Humans; D007167:Immunotherapy; D018979:Myositis, Inclusion Body / Q000453:epidemiology / Q000276:immunology / Q000473:pathology* / Q000628:therapy*; D009419:Nerve Tissue Proteins / Q000502:physiology; D009465:Neuromuscular Agents / Q000627:therapeutic use
chemical_list: D009419:Nerve Tissue Proteins; D009465:Neuromuscular Agents