Inflammatory myopathies: disease mechanisms.
OBJECTIVE
Recent developments pertaining to disease mechanisms in the inflammatory myopathies are discussed, emphasizing those areas that are of particular interest to me.


RESULTS
The identification and further characterization of the type 1 interferon pathway in dermatomyositis is leading down a path of genomic medicine. Myonuclear structural abnormalities and the presence of nucleic acid-binding proteins, including the TAR DNA binding protein TDP-43, in sporadic inclusion body myositis (sIBM) sarcoplasm are important recent observations. This is an area likely to provide deep understanding of the mechanism of myofiber injury in sIBM. Proteomic characterization of proteins in sIBM muscle, muscle functioning as a lymphoid tissue, and the nature of belief systems, particularly one pertaining to beta-amyloid and sIBM, are other areas of interest.


CONCLUSIONS
Clarification of disease mechanisms is providing a basis for rational drug development for some patients with myositis.
Greenberg
Steven A
SA
Children's Hospital Informatics Program, Department of Neurology, Division of Neuromuscular Disease, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA. sagreenberg@partners.org
D000682:Amyloid / Q000235:genetics; D001284:Atrophy; D004268:DNA-Binding Proteins / Q000235:genetics; D003882:Dermatomyositis / Q000209:etiology* / Q000235:genetics / Q000473:pathology*; D020022:Genetic Predisposition to Disease; D006801:Humans; D007370:Interferon Type I / Q000235:genetics; D018482:Muscle, Skeletal / Q000473:pathology*; D018979:Myositis, Inclusion Body / Q000209:etiology* / Q000235:genetics
D000682:Amyloid; D004268:DNA-Binding Proteins; D007370:Interferon Type I