title: Sporadic inclusion body myositis: new insights and potential therapy.
abstract: OBJECTIVE
  To describe new insights and developments in the pathogenesis, diagnosis and treatment of sporadic inclusion body myositis (IBM).
  
  
  RESULTS
  Various hypothesis about the pathogenesis of IBM continue to be investigated, including autoimmune factors, mitochondrial dysfunction, protein dyshomeostasis, altered nucleic acid metabolism, myonuclear degeneration and the role of the myostatin pathway. Serum autoantibodies against cytosolic 5'-nucleotidase 1A have been identified in IBM showing moderate diagnostic performance. The differential diagnostic value of histopathological features, including different protein aggregates, continues to be evaluated. MRI may also be of monitoring value in IBM. New therapeutic strategies are being tested in IBM patients, namely the upregulation of the heat shock response and the antagonism of myostatin.
  
  
  CONCLUSIONS
  Recent important advances have occurred in IBM. These advances, including recent and ongoing clinical trials, may lead to earlier diagnosis and improved understanding and treatment of the disease. Despite improved knowledge, IBM continues to be a puzzling disease and the pathogenesis remains to be clarified. An interdisciplinary, bench to bedside translational research approach is crucial for the successful identification of novel treatments for this debilitating, currently untreatable disorder.
authors.affiliation: aMRC Centre for Neuromuscular Diseases, University College London, London, UK bDepartment of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA.
authors.forename: Pedro M
authors.initials: PM
authors.lastname: Machado
authors.forename: Mazen M
authors.initials: MM
authors.lastname: Dimachkie
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Barohn
mesh_terms: D015720:5'-Nucleotidase / Q000276:immunology; D000906:Antibodies / Q000097:blood; D006801:Humans; D008279:Magnetic Resonance Imaging; D018979:Myositis, Inclusion Body / Q000097:blood / Q000175:diagnosis* / Q000628:therapy*
chemical_list: D000906:Antibodies; D015720:5'-Nucleotidase