IDIOPATHIC INFLAMMATORY MYOPATHIES. A REVIEW

Miopatías inflamatorias idiopáticas: una mirada actualizada al diagnóstico y el manejo

Author
Acosta, Ignacio; Clínica Alemana Santiago -Universidad del Desarrollo

Matamala, José Manuel; Departamento Neurología y Psiquiatría, Clínica Alemana de Santiago-Universidad del Desarrollo, Santiago, Chile Departamento de Ciencias Neurológicas, Facultad de Medicina, Universidad de Chile, Santiago, Chile Instituto Milenio de Neurociencia Biomédica (BNI), Facultad de Medicina, Universidad de Chile, Santiago, Chile

Jara, Paula; Departamento Neurología y Psiquiatría, Clínica Alemana de Santiago-Universidad del Desarrollo, Santiago, Chile.

Pino, Francisca; Departamento Neurología y Psiquiatría, Clínica Alemana de Santiago-Universidad del Desarrollo, Santiago, Chile.

Gajardo, Alejandra; Departamento de Anatomía Patológica, Clínica Alemana de Santiago-Universidad del Desarrollo, Santiago, Chile.

Verdugo, Renato; Departamento Neurología y Psiquiatría, Clínica Alemana de Santiago-Universidad del Desarrollo, Santiago, Chile

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http://www.revistamedicadechile.cl/ojs/index.php/rmedica/article/view/6735
Abstract
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 clinical pictures, 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.
 
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 clinical pictures, 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.
 
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