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Dermatomyositis, polymyositis and immune-mediated necrotising myopathies
Journal article   Open access   Peer reviewed

Dermatomyositis, polymyositis and immune-mediated necrotising myopathies

Y-B Luo and F.L. Mastaglia
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Vol.1852(4), pp.622-632
2015
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Abstract

Dermatomyositis, polymyositis and immune-mediated necrotising myopathy are major forms of idiopathic inflammatory myopathy. We review here recent developments in understanding the pathology and pathogenesis of these diseases, and characterisation of autoantibody biomarkers. Dermatomyositis is traditionally considered to be due to a complement-mediated microangiopathy but the factors responsible for complement activation remain uncertain. Recent studies have emphasised the importance of the type I interferon pathway in the pathogenesis of the disease and have identified autoantibodies with specificities for different clinical subgroups of patients. Polymyositis is characterised by a cytotoxic T cell response targeting as yet unidentified muscle antigens presented by MHC Class I molecules, and can occur in isolation but is more often part of a multi-systemic overlap syndrome. The immune-mediated necrotising myopathies are heterogeneous and are distinguished from polymyositis by the sparseness of inflammatory infiltrates and recognition of an association with specific autoantibodies such as anti-SRP and anti-HMGCR in many cases.

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Collaboration types
Domestic collaboration
International collaboration
Citation topics
1 Clinical & Life Sciences
1.106 Rheumatology
1.106.1684 Dermatomyositis
Web Of Science research areas
Biochemistry & Molecular Biology
Biophysics
Cell Biology
ESI research areas
Clinical Medicine
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