title: Inflammatory muscle diseases: a critical review on pathogenesis and therapies.
abstract: Based on unique clinicopathological criteria, the most common immune inflammatory muscle disorders include Dermatomyositis (DM), Polymyositis (PM), Necrotizing Myositis (NM), and sporadic Inclusion Body Myositis (sIBM). DM is an undeniably a complement-mediated microangiopathy with destruction of capillaries, hypoperfusion, and inflammatory cell stress on the perifascicular regions. Necrotizing Myopathy is a poorly studied subacute myopathy triggered by toxic, viral, or autoimmune factors with macrophages as the final effector cells. In PM and IBM cytotoxic CD8-positive T-cells clonally expand in situ and invade MHC-I-expressing muscle fibers. In sIBM, in addition to autoimmune inflammation, there are degenerative features characterized by vacuolization and accumulation of stressor and amyloid-related molecules. Advances in the immunobiology of these disorders are discussed including the interaction between pro-inflammatory and beta-amyloid or stressor proteins. A critical review regarding tissue biomarkers and strategies for more effective treatments are presented.
authors.affiliation: Department of Neurosciences, Imperial College, London, UK. m.dalakas@imperial.ac.uk
authors.forename: Marinos C
authors.initials: MC
authors.lastname: Dalakas
mesh_terms: D000818:Animals; D015415:Biomarkers / Q000378:metabolism; D003882:Dermatomyositis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007249:Inflammation / Q000209:etiology / Q000276:immunology / Q000628:therapy; D009220:Myositis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D018979:Myositis, Inclusion Body / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D017285:Polymyositis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*
chemical_list: D015415:Biomarkers