Inclusion body myositis: old and new concepts.
Inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy occurring in patients over the age of 50 years and probably accounts for about 30% of all inflammatory myopathies. Muscle biopsy characteristically reveals endomysial inflammation, small groups of atrophic fibres, eosinophilic cytoplasmic inclusions and muscle fibres with one or more rimmed vacuoles. However, any given biopsy may lack these histopathological abnormalities; the clinical examination is often the key to diagnosis. Early and often asymmetrical weakness and atrophy of the quadriceps and flexor forearm muscles (ie, wrist and finger flexors) are the clinical hallmarks of IBM. The pathogenesis of IBM is unknown. It may be autoimmune inflammatory myopathy or a primary degenerative myopathy with a secondary inflammatory. A prevailing theory is that there is an overproduction of beta-amyloid precursor protein in muscle fibres that is somehow cleaved into abnormal beta-amyloid, and the accumulation of the latter is somehow toxic to muscle fibres. However, there are many problems with this theory and more work needs to be done. Unfortunately, IBM is generally refractory to therapy. Further research into the pathogenesis, along with both preliminary small pilot trials and larger double blind, placebo controlled efficacy trials, are needed to make progress in our understanding and therapeutic approach for this disorder.
Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis St, Boston, MA 02115, USA. aamato@partners.org
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Amato
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Barohn
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis; D003937:Diagnosis, Differential; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018979:Myositis, Inclusion Body / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D017285:Polymyositis / Q000175:diagnosis; D011379:Prognosis; D040901:Proteomics
D007166:Immunosuppressive Agents