Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies

@article{Dalakas2006SporadicIB,
  title={Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies},
  author={Marinos C. Dalakas},
  journal={Nature Clinical Practice Neurology},
  year={2006},
  volume={2},
  pages={437-447}
}
  • M. Dalakas
  • Published 1 August 2006
  • Biology, Medicine
  • Nature Clinical Practice Neurology
Sporadic inclusion body myositis (sIBM) presents with a characteristic clinical phenotype of slow-onset weakness and atrophy, affecting proximal and distal limb muscles and facial and pharyngeal muscles. Histologically, sIBM is characterized by chronic myopathic features, lymphocytic infiltrates invading non-vacuolated fibers, vacuolar degeneration, and accumulation of amyloid-related proteins. The cause of sIBM is unclear, but two processes—one autoimmune and the other degenerative—appear to… 
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TLDR
It is proposed that the disproportionate involvement of certain muscles in sIBM may be due to the existence of muscle group-specific transcriptomes which are differentially affected by the disease process and that the male predominance of the disease may indicate the influence of genes preferentially expressed in males.
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Figures that show abnormal localization of nuclear proteins associated with MAP kinase in s-IBM muscle fibers suggest that inhibition of nuclear transport during myogenesis is suggested and the theory that nuclear damage is closely associated with its etiology is supported.
Idiopathic Inflammatory Myopathies: A Review of Immunopathological Features and Current Models of Pathogenesis
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The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of chronic systemic disorders characterized by muscle inflammation and progressive muscle weakness, and a growing body of evidence suggests that genetically susceptible individuals probably develop an idiopathy in response to particular environmental stimuli.
Mitochondrial Biology in Sporadic Inclusion Body Myositis
TLDR
The role of the mitochondria in the development of sIBM and the role of amyloid beta on mitochondrial function in skeletal muscle are evaluated to help to identify novel prevention and/ or treatment strategies.
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TLDR
Understanding the paradoxes in sporadic inclusion body myositis is important in determining rational therapy for the disease.
Pathogenesis and therapies of immune-mediated myopathies.
  • M. Dalakas
  • Biology, Medicine
    Autoimmunity reviews
  • 2012
Pathophysiology of inflammatory and autoimmune myopathies.
Sporadic inclusion body myositis: an unsolved mystery.
TLDR
It remains uncertain whether sIBM is primarily an immune-mediated inflammatory myopathy or a degenerative myopathy with an associated inflammatory component.
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