title: Idiopathic inflammatory myopathies.
abstract: The idiopathic inflammatory myopathies (IIM) consist of rare heterogeneous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis. Despite many similarities, the IIM are fairly heterogeneous from the histopathologic and pathogenetic standpoints, and also show some clinical and treatment-response differences. The field has witnessed significant advances in our understanding of the pathophysiology and treatment of these rare disorders. This review focuses on dermatomyositis, polymyositis, and necrotizing myopathy, and examines current and promising therapies.
authors.affiliation: Neurophysiology Division, Neuromuscular Section, Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mail Stop 2012, Kansas City, KS 66160, USA. Electronic address: mdimachkie@kumc.edu.
authors.forename: Mazen M
authors.initials: MM
authors.lastname: Dimachkie
authors.affiliation: Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mail Stop 2012, Kansas City, KS 66160, USA.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Barohn
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.
authors.forename: Anthony A
authors.initials: AA
authors.lastname: Amato
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis; D003882:Dermatomyositis / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D018908:Muscle Weakness / Q000150:complications; D009220:Myositis / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D018979:Myositis, Inclusion Body / Q000175:diagnosis
keywords: Clinical presentation; Dermatomyositis; Diagnosis; Inclusion body myositis; Necrotizing myopathy; Pathology; Polymyositis; Treatment