Barsotti Simone, Lundberg Ingrid E
a Rheumatology Unit, Department of Clinical and Experimental Medicine , University of Pisa , Pisa Italy and Department of Medical Biotechnology, University of Siena, Siena, Italy.
b Division of rheumatology, Department of Medicine , Solna, Karolinska Institutet, and Karolinska University Hospital , Stockholm , Sweden.
Immunol Med. 2018 Jun;41(2):46-54. doi: 10.1080/13497413.2018.1481571. Epub 2018 Sep 11.
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.
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,IIMs被分为多发性肌炎、皮肌炎和包涵体肌炎,但这种分类有几个局限性,因为这三个IIM亚组的临床特征以及治疗反应各不相同。在过去几年中,已在IIMs患者中鉴定出几种新型自身抗体。这些自身抗体可以是肌炎特异性自身抗体(MSAs)或肌炎相关自身抗体(MAAs),它们可能会带来一种IIMs分类的新方法。这种新方法有助于将患者细分为更同质化的群体,因为患者出现多种MSAs阳性的情况非常罕见,而且每种自身抗体通常都与特定的临床特征相关。此外,MSAs还可以帮助识别没有肌肉症状的IIMs亚组,比如以皮肤表现、关节炎或间质性肺病为主要临床特征的患者。此外,由于一些自身抗体可能与明显严重的表现有关,如癌症或快速进展的间质性肺病,它们还可以对患者进行预后分层。