• Alain Meyer, Béatrice Lannes, Joëlle Goetz, Andoni Echaniz-Laguna, Dan Lipsker, Laurent Arnaud, Thierry Martin, Jacques Eric Gottenberg, Bernard Geny, and Jean Sibilia.
• Service de physiologie et d'explorations fonctionnelles, hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France; Service de rhumatologie, hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France; Centre de référence des maladies auto-immunes rares, hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France; Fédération de médecine translationnelle de Strasbourg, université de Strasbourg, 67000 Strasbourg, France. Electronic address: alain.meyer1@chru-strasbourg.fr.
• Joint Bone Spine. 2018 Jan 1; 85 (1): 23-33.

AbstractGreater accuracy in clinical descriptions combined with advances in muscle histology and immunology have established that inflammatory myopathies (IMs), similarly to inflammatory rheumatic diseases, constitute a highly heterogeneous group of conditions. The topographic distribution, severity, and tempo of onset of the myopathy vary widely, and the histological findings distinguish at least five different profiles, which may reflect different pathophysiological processes. Most IMs are connective tissue diseases that can affect multiple organs, among which the most common targets are the skin, joints, and lungs. The extramuscular manifestations may antedate the muscular involvement and should therefore suggest a diagnosis of IM even in the absence of obvious muscle disease. About 20 different autoantibodies have been identified in patients with IM. Some are mutually exclusive and associated with specific combinations of clinical manifestations. Following the model of antisynthetase syndrome, about 10 syndromes associated with autoantibodies specific of IM have been identified. Thus, polymyositis is now emerging as a rare entity that is often mistaken for more recently described patterns of IM. No consensus exists to date about the classification of IMs. Nevertheless, the clinical manifestations, autoantibody profile, and muscle histology can be used to distinguish patient subgroups with fairly homogeneous patterns of complications, treatment responses, and outcomes. These subgroups are also characterized by specific genetic and environmental factors. The advances made in the nosology of IMs have benefited the diagnosis, personalization of treatment strategies, and understanding of pathophysiological mechanisms. They can be expected to assist in the development of specific treatments.Copyright © 2017 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

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