Article information
Abstract
Dermatomyositis is an idiopathic inflammatory myopathy that affects skeletal muscle and the skin. Idiopathic inflammatory myopathies are characterized by the production of autoantibodies directed against different cell structures. Some of these autoantibodies are specific to idiopathic inflammatory myopathies (myositis-specific antibodies) whereas others are found in a range of overlap syndromes (myositis-associated antibodies). Although they are all associated with certain clinical and physiopathological characteristics of myositis, myositis-specific antibodies are essentially the most useful markers for clinical diagnosis, classification, and prognosis in idiopathic inflammatory myopathies. In recent years, two new myositis-specific antibodies–-in clinically amyopathic dermatomyositis (CADM), CADM-140 and, in cancer-associated dermatomyositis, anti-p155/p140–-have been identified. This is of great importance as no myositis-specific antibodies had previously been detected in these clinical subgroups. The identification of target antigens that are recognized by these antibodies is essential for a better understanding of the pathogenesis of these diseases.
Key words:
idiopathic inflammatory myopathy
polymyositis
amyopathic dermatomyositis
cancer-associated dermatomyositis
myositis-specific antibodies
Resumen
La dermatomiositis es un tipo de miopatía inflamatoria idiopática que afecta al músculo esquelético y a la piel. Las miopatías inflamatorias idiopáticas se caracterizan por la producción de autoanticuerpos que se dirigen frente a diversas estructuras de la célula. Algunos de estos autoanticuerpos resultan específicos de las miopatías inflamatorias idiopáticas (anticuerpos específicos de miositis), mientras que otros se encuentran en diversos síndromes de solapamiento (anticuerpos asociados a miositis). Todos ellos determinan ciertas características clínicas y patofisiológicas de miositis, pero son principalmente los anticuerpos específicos de miositis los que constituyen marcadores muy útiles para el diagnóstico clínico, la clasificación y la predicción del pronóstico en las miopatías inflamatorias idiopáticas. En los últimos años se han identificado dos nuevos anticuerpos específicos de miositis, el anti-CADM-140 en la dermatomiositis amiopática y el anti-p155 y anti-p155/p140 en la dermatomiositis asociada a cáncer. Ello tiene una gran trascendencia ya que en estos subgrupos clínicos no se detectaban anticuerpos específicos de miositis. La identificación de los antígenos diana que reconocen estos anticuerpos resultará fundamental para comprender mejor la etiopatogenia de estas enfermedades.
Palabras clave:
miopatía inflamatoria idiopática
polimiositis
dermatomiositis amiopática
dermatomiositis asociada a cáncer
anticuerpos específicos de miositis
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