成人炎症性肌病的分类和管理。
Classification and management of adult inflammatory myopathies.
机构信息
Systemic Autoimmune Diseases Unit, Vall d'Hebron General Hospital, Universitat Autònoma de Barcelona, Barcelona, Spain.
Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
出版信息
Lancet Neurol. 2018 Sep;17(9):816-828. doi: 10.1016/S1474-4422(18)30254-0.
Inflammatory myopathies, collectively known as myositis, are heterogeneous disorders characterised by muscle inflammation, and frequently accompanied by extramuscular manifestations that affect the skin, lung, and joints. Patients with inflammatory myopathies were previously classified as having dermatomyositis if characteristic rashes accompanied the muscle involvement, and as having polymyositis if no rashes were present. Five main types of inflammatory myopathies are now widely recognised: dermatomyositis, immune-mediated necrotising myopathy, sporadic inclusion-body myositis, overlap myositis (including antisynthetase syndrome), and polymyositis. The discovery of autoantibodies that are specifically associated with characteristic clinical phenotypes has been instrumental to the understanding of inflammatory myopathies. Treatment is still largely based on expert opinion, but several studies have shown effectiveness of different therapies in various subsets of inflammatory myopathies. These advances will undoubtedly improve the outcomes of patients with inflammatory myopathies.
炎性肌病，统称为肌炎，是一组异质性疾病，其特征为肌肉炎症，并常伴有肌肉外表现，影响皮肤、肺和关节。以前，如果肌肉受累伴有特征性皮疹，则将炎性肌病患者归类为皮肌炎，如果没有皮疹，则归类为多发性肌炎。现在广泛认识到五种主要类型的炎性肌病：皮肌炎、免疫介导的坏死性肌病、特发性包涵体肌炎、重叠性肌炎（包括抗合成酶综合征）和多发性肌炎。发现与特征性临床表型特异性相关的自身抗体对理解炎性肌病至关重要。治疗仍然主要基于专家意见，但多项研究表明，不同疗法对炎性肌病的不同亚组有效。这些进展无疑将改善炎性肌病患者的预后。
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