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[炎性肌病。皮肌炎、多发性肌炎和包涵体肌炎]

[Inflammatory myopathies. Dermatomyositis, polymyositis, and inclusion body myositis].

作者信息

Selva O'Callaghan Albert, Trallero Araguás Ernesto

机构信息

Servicio de Medicina Interna. Hospital General Universitario Vall d'Hebron. Barcelona. España.

出版信息

Reumatol Clin. 2008 Sep;4(5):197-206. doi: 10.1016/S1699-258X(08)72464-1. Epub 2008 Nov 18.

Abstract

Idiopathic inflammatory myopathies are a group of heterogeneous, acquired systemic diseases characterized by progressive symmetrical muscle weakness, elevated serum levels of muscle enzymes, electromyographic abnormalities, and inflammatory infiltrates on muscle biopsy. Characteristic histopathologic features allow classification of idiopathic inflammatory myopathies into polymyositis, dermatomyositis, and sporadic inclusion-body myositis. These are commonly regarded as autoimmune disorders, and various autoantibodies directed to specific nuclear and cytoplasmic antigens are found. Other organs besides the muscle can be involved being the skin and lung the most frequent. Occasionally dermatomyositis and polymyositis can be associated with cancer in a paraneoplastic manner. Corticosteroids and immunosuppressive agents are the mainstay therapy, although in refractory cases biologic therapy can be used. Physical therapy can not be forgotten.

摘要

特发性炎性肌病是一组异质性的获得性全身性疾病,其特征为进行性对称性肌无力、血清肌酶水平升高、肌电图异常以及肌肉活检有炎性浸润。特征性组织病理学特征可将特发性炎性肌病分为多发性肌炎、皮肌炎和散发性包涵体肌炎。这些疾病通常被视为自身免疫性疾病,并且可发现针对特定核抗原和胞质抗原的各种自身抗体。除肌肉外,其他器官也可受累,最常见的是皮肤和肺。偶尔,皮肌炎和多发性肌炎可呈副肿瘤性方式与癌症相关。皮质类固醇和免疫抑制剂是主要治疗方法,尽管在难治性病例中可使用生物治疗。物理治疗也不容忽视。

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