Update in inclusion body myositis.
OBJECTIVE
The purpose of this study is to review recent scientific advances relating to the natural history, cause, treatment and serum and imaging biomarkers of inclusion body myositis (IBM).


RESULTS
Several theories regarding the aetiopathogenesis of IBM are being explored and new therapeutic approaches are being investigated. New diagnostic criteria have been proposed, reflecting the knowledge that the diagnostic pathological findings may be absent in patients with clinically typical IBM. The role of MRI in IBM is expanding and knowledge about pathological biomarkers is increasing. The recent description of autoantibodies to cytosolic 5' nucleotidase 1A in patients with IBM is a potentially important advance that may aid early diagnosis and provides new evidence regarding the role of autoimmunity in IBM.


CONCLUSIONS
IBM remains an enigmatic and often misdiagnosed disease. The pathogenesis of the disease is still not fully understood. To date, pharmacological treatment trials have failed to show clear efficacy. Future research should continue to focus on improving understanding of the pathophysiological mechanisms of the disease and on the identification of reliable and sensitive outcome measures for clinical trials. IBM is a rare disease and international multicentre collaboration for trials is important to translate research advances into improved patient outcomes.
MRC Centre for Neuromuscular Diseases, Institute of Neurology, University College London, London, UK *Pedro Machado and Stefen Brady have contributed equally to this article.
Pedro
P
Machado
Stefen
S
Brady
Michael G
MG
Hanna
D015720:5'-Nucleotidase / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D018979:Myositis, Inclusion Body / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy
D001323:Autoantibodies; D015415:Biomarkers; D015720:5'-Nucleotidase; C552626:NT5C1A protein, human