Acosta Ignacio, Matamala José Manuel, Jara Paula, Pino Francisca, Gallardo Alejandra, Verdugo Renato
Departamento Neurología y Psiquiatría, Clínica Alemana de Santiago, Universidad del Desarrollo, Santiago, Chile.
Departamento de Anatomía Patológica, Clínica Alemana de Santiago, Universidad del Desarrollo, Santiago, Chile.
Rev Med Chil. 2019 Mar;147(3):342-355. doi: 10.4067/S0034-98872019000300342.
Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired immune-mediated diseases, which typically involve the striated muscle with a variable involvement of the skin and other organs. Clinically, they are characterized by proximal muscle weakness, elevation of muscle enzymes, myopathic changes on electromyography and an abnormal muscle biopsy. The different IIM have been classified according to their distinctive histopathologic features in dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). Several myositis-specific antibodies are associated with the different phenotypes, as well as with different risk of neoplastic disease and systemic complications. The basis for the treatment of DM, PM, and IMNM is immunosuppression. For IBM there are only symptomatic treatments. Steroids, associated or not with other immunosuppressant drugs, are the first line of treatment. Biologic drugs will allow future individualized therapies. The 10-year survival of DM, PM and IMNM is 62 to 90%. The leading causes of death are neoplastic, lung and cardiac complications. IBM does not impair survival, although it affects the quality of life.
特发性炎性肌病（IIM）是一组异质性的获得性免疫介导疾病，通常累及横纹肌，皮肤和其他器官也可不同程度受累。临床上，其特征为近端肌无力、肌酶升高、肌电图显示肌病改变以及肌肉活检异常。不同的IIM已根据其在皮肌炎（DM）、多发性肌炎（PM）、包涵体肌炎（IBM）和免疫介导坏死性肌病（IMNM）中的独特组织病理学特征进行分类。几种肌炎特异性抗体与不同的表型相关，也与肿瘤性疾病和全身并发症的不同风险相关。DM、PM和IMNM的治疗基础是免疫抑制。对于IBM，只有对症治疗。类固醇，无论是否与其他免疫抑制药物联合使用，都是一线治疗药物。生物药物将使未来的治疗能够实现个体化。DM、PM和IMNM的10年生存率为62%至90%。主要死亡原因是肿瘤、肺部和心脏并发症。IBM虽不影响生存率，但会影响生活质量。
