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Las miopatías inflamatorias tienen un riesgo aumentado de malignidad, siendo la dermatomiositis la de mayor asociación. La neoplasia puede ser sólida o hematológica. Existen factores clínicos y de laboratorio que pueden aumentar o disminuir el riesgo de presentación de miositis como enfermedad paraneoplásica. Entre los principales factores de riesgo, se ha observado la presencia de anticuerpos anti-TIF1, el anti-NXP2 y en menor medida el anti-SAE. La principal teoría de su origen apunta hacia la respuesta inmune derivada de la creación de proteínas mutadas por parte de las células cancerosas. El diagnóstico de malignidad se puede realizar antes, durante o después del diagnóstico de dermatomiositis. No existe un régimen terapéutico establecido, sin embargo se ha propuesto el uso de esteroides, inmunosupresores o inmunoglobulinas, así como la terapia efectiva contra el cáncer.
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