Fibrosis sistémica nefrogénica: Presentación de un caso y revisión de la literatura / Nephrogenic systemic fibrosis: A case report and review of the literature

RESUMEN

La fibrosis sistémica nefrogénica (FSN) es una enfermedad fibrosante de reciente identificación, que afecta exclusivamente a pacientes con insuficiencia renal. Se caracteriza por una fibrosis extensa de los tejidos, especialmente de la piel. Aunque su etiología no es del todo conocida, se ha asociado fuertemente al uso de contrastes de gadolinio en pacientes con insuficiencia renal avanzada. Se describe a continuación un caso de FSN diagnosticado en España.

ABSTRACT

Nephrogenic systemic fibrosis (NSF) is a rare condition appearing only in patients with severe renal impairment and characterized by widespread tissue fibrosis. Although the pathogenesis of this disorder remains unclear, there is evidence of a strong association between gadolinium-based contrast agents (Ga-CA) exposure and the triggering of this disease. Cellular elements involved in pathogenesis of NSF include bone-marrow-derived collagen producing fibrocytes, myofibroblasts and activated macrophages. We present the case of a caucasian female with advanced renal failure who underwent several times magnetic resonance imaging (MRI) procedures using Ga-CA. The patient developed a progressive fibrosing disorder with bilateral indurated papules on the lower legs, joint contractures and hairlessness, and was diagnosed of NSF based on the histologic findings of skin biopsies. Later in the disease she presented with systemic involvement and fatal evolution.