Shimizu Jun
Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Brain Nerve. 2010 Apr;62(4):427-32.
Inflammatory myopathies, also referred to as myositis, are a heterogeneous group of chronic inflammatory muscle diseases characterized by various clinical features and histological changes; in addition, patient with these disease exhibit positivity for autoantibodies as well as progressive inflammatory muscle damage and experienced weakness. Although it has been well known for a century that myositis, particularly dermatomyositis, can be associated with malignancy, it was not until recently that the result of robust epidemiological studies confirmed this association. Malignancy-associated myositis differs from primary myositis in many aspects. Prognosis and life expectancy for patients are determined on the basis of the underlying malignancy. Therefore, patient-specific examinations to detect an underlying cancer are important for the management of these patients. Recently, a novel myositis-specific autoantibody (anti-p155 or p155/p140 antibody) was identified in malignancy-associated myositis. The discovery of this autoantibody is important not only for an early diagnosis of adult-onset myositis patients with a higher risk of malignancy but also for a better understanding of the pathogenesis of paraneoplastic myositis. In addition, it has also been recently found that both regenerating cells in myositis muscles and several cancers known to be associated with myositis express high levels of myositis-specific autoantigens. Therefore, a model of paraneoplasia focusing on the expression of common autoantigens expression and immuno-targeting between cancer and muscle tissues in myositis has been proposed. In this review, we aim to describe epidemiological evidence for an association between myositis and malignancy, and to describe the clinical features of malignancy-associated myositis. We also aim to focus on a recently proposed model to understand the development of malignancy-associated myositis.
炎性肌病,也称为肌炎,是一组异质性的慢性炎性肌肉疾病,具有各种临床特征和组织学变化;此外,这些疾病的患者表现出自身抗体阳性以及进行性炎性肌肉损伤和肌无力。尽管一个世纪以来人们就已经熟知肌炎,尤其是皮肌炎,可能与恶性肿瘤有关,但直到最近,强有力的流行病学研究结果才证实了这种关联。恶性肿瘤相关肌炎在许多方面与原发性肌炎不同。患者的预后和预期寿命取决于潜在的恶性肿瘤。因此,针对特定患者进行检测以发现潜在癌症对于这些患者的管理很重要。最近,在恶性肿瘤相关肌炎中发现了一种新型的肌炎特异性自身抗体(抗p155或p155/p140抗体)。这种自身抗体的发现不仅对于早期诊断具有较高恶性肿瘤风险的成人起病型肌炎患者很重要,而且对于更好地理解副肿瘤性肌炎的发病机制也很重要。此外,最近还发现,肌炎肌肉中的再生细胞以及几种已知与肌炎相关的癌症都表达高水平的肌炎特异性自身抗原。因此,有人提出了一种副肿瘤形成模型,该模型侧重于肌炎中癌症与肌肉组织之间共同自身抗原表达和免疫靶向的表达。在这篇综述中,我们旨在描述肌炎与恶性肿瘤之间关联的流行病学证据,并描述恶性肿瘤相关肌炎的临床特征。我们还旨在关注最近提出的一个模型,以了解恶性肿瘤相关肌炎的发展。