Inflammatory myopathies are chronic, immune-mediated diseases characterised by progressive proximal muscle weakness. They encompass a variety of syndromes with protean manifestations. The diagnosis is based on Bohan and Peter's classification criteria, which nowadays seem to be obsolete. Our increasing knowledge about the risk factors, genetic susceptibility and immunological pathways in the disease mechanism leads to the establishment of a new, immunogenetically and serologically validated diagnostic criteria system. The treatment of idiopathic inflammatory myopathy is also a complex task requiring much experience. The aims of therapy are to increase muscle strength, prevent the development of contractures and manage the systemic manifestations of the disease. The most important one is the early detection of diseases and patients' immunological control in special centres. Using the basis therapeutic drugs temporary or permanent remission can be achieved, which improves patients' quality of life and functional ability. Rehabilitation and physiotherapy in the remission period may significantly improve the outcome of patients with functional disorders. The introduction of new biological therapies further allows us to control the myosins patients' state more effectively. The aim of this review is to summarise our knowledge about clinical symptoms, pathomechanism, as well as genetic, serologic and environmental risk factors. We would also like to present the way to diagnosis and the latest research about diagnostic criteria system, proposed outcome measures and therapeutic possibilities. (C) 2012 Elsevier Ltd. All rights reserved.
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Univ Kansas, Med Ctr, Dept Neurol, Neurophysiol Div,Neuromuscular Sect, Kansas City, KS 66160 USAUniv Kansas, Med Ctr, Dept Neurol, Neurophysiol Div,Neuromuscular Sect, Kansas City, KS 66160 USA
Dimachkie, Mazen M.
Barohn, Richard J.
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Univ Kansas, Med Ctr, Dept Neurol, Kansas City, KS 66160 USAUniv Kansas, Med Ctr, Dept Neurol, Neurophysiol Div,Neuromuscular Sect, Kansas City, KS 66160 USA
Barohn, Richard J.
Amato, Anthony A.
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Brigham & Womens Hosp, Harvard Med Sch, Dept Neurol, Boston, MA 02115 USAUniv Kansas, Med Ctr, Dept Neurol, Neurophysiol Div,Neuromuscular Sect, Kansas City, KS 66160 USA