Myositis an evolving spectrum of disease.
Myositis an evolving spectrum of disease.
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DOI：
10.1080/13497413.2018.1481571
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发表时间：
2018-06-01
影响因子：
4.4
通讯作者：
Lundberg, Ingrid E
中科院分区：
文献类型：
--
作者：
Barsotti, Simone;Lundberg, Ingrid E
特发性炎性肌病（IIMs）是一组异质性疾病，其共同特征是骨骼肌炎症和肌无力。传统上，IIM被细分为多发性肌炎、皮肌炎和包涵体肌炎，但这种细分有几个局限性，因为三个IIM亚组的临床特征和治疗反应各不相同。在过去的几年中，一些新的自身抗体的患者与IIM已被确定。这些自身抗体可以是肌炎特异性自身抗体（MSA）或肌炎相关自身抗体（MAA），它们可能导致一种新的方法来分类IIM。这种新方法可以帮助将患者细分为更同质的组，因为患者具有一种以上的MSA阳性非常罕见，并且每种自身抗体通常与特定的临床特征相关。此外，MSA可以帮助识别没有肌肉症状的IIM子集，如皮肤表现，关节炎或间质性肺病代表主要临床特征的患者。此外，由于一些自身抗体可能与显著严重的表现相关，例如癌症或快速进展的间质性肺病，因此它们也可以提供患者的预后分层。
The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of disorders characterized, as common feature, by inflammation of skeletal muscle and muscle weakness. Traditionally, IIMs have been subclassified in into polymyositis, dermatomyositis and inclusion body myositis, but this subclassification has several limitations, because clinical features as well as treatment response vary within the three IIM subgroups. In the last years several novel autoantibodies in patients with IIMs have been identified. These autoantibodies can be myositis-specific autoantibodies (MSAs) or myositis-associated autoantibodies (MAAs) and they may lead to a new approach to the classification of IIMs. This novel approach could help to subdivide patients in more homogeneous groups because, it is very rare that a patient has more than one MSAs positivity and each autoantibody is frequently associated with specific clinical features. Moreover, MSAs can help to identify subsets of IIMs also without muscular symptoms, like patients in which skin manifestations, arthritis or interstitial lung disease represent the main clinical feature. Additionally, as some autoantibodies may be associated to markedly severe manifestations, such as cancer or rapidly progressive interstitial lung disease, they can also provide a prognostic stratification of the patients.