Idiopathic inflammatory myopathies. A review

Ignacio Acosta; José Manuel Matamala; Paula Jara; Francisca Pino; Alejandra Gallardo; Renato Verdugo

Keywords: dermatomyositis, polymyositis, myositis, Inclusion Body

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 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.

Más información

Título según WOS: Idiopathic inflammatory myopathies. A review
Título según SCOPUS: Idiopathic inflammatory myopathiea review [Miopatías inflamatorias idiopáticas: Una mirada actualizada al diagnóstico y el manejo]
Título según SCIELO: Miopatías inflamatorias idiopáticas: una mirada actualizada al diagnóstico y el manejo
Título de la Revista: REVISTA MEDICA DE CHILE
Volumen: 147
Número: 3
Editorial: SOC MEDICA SANTIAGO
Fecha de publicación: 2019
Página de inicio: 342
Página final: 355
Idioma: Spanish
DOI:

10.4067/S0034-98872019000300342

Notas: ISI, SCIELO, SCOPUS