Manejo de la Esclerosis Sistémica Cutánea Limitada y Difusa en Dermatología
La esclerosis sistémica (SSc: systemic sclerosis cutaneous) es una enfermedad rara, autoinmune y sistémica. En ella se produce una disfunción vascular generalizada y una alteración del sistema inmunológico, que en un sujeto genéticamente predispuesto e interviniendo factores ambientales que aún son desconocidos, dan lugar a una fibrosis progresiva pudiendo afectar a múltiples órganos.
La esclerosis sistémica es una enfermedad rara, con una incidencia de 2 a 10 casos por millón de habitantes y año, y afecta más frecuentemente a mujeres que a hombres con una relación de 5-9:1. Además, los pacientes tienen una clínica muy heterogénea, y por todo ello es muy complicado diseñar ensayos clínicos y comparar diferentes fármacos entre sí.
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