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Vol. 16. Issue 4.
Pages 361-373 (December 2009)
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Vol. 16. Issue 4.
Pages 361-373 (December 2009)
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Fibrosis retroperitoneal idiopática: un enigma médico
Retroperitoneal fibrosis: a medical enigma
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Publio Giovanni Saavedra Ramírez1, Marcos Arango Barrientos2, Luis Alonso González Naranjo3
1 Médico internista-reumatólogo, Hospital Universitario San Vicente de Paúl, Medellín
2 Médico residente de medicina interna, Universidad de Antioquia-Hospital Universitario San Vicente de Paúl, Medellín
3 Profesor Sección de Reumatología, Hospital Universitario San Vicente de Paúl, Universidad de Antioquia. Medellín, Colombia
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Resumen

La fibrosis retroperitoneal es una enfermedad rara de causa desconocida. Fue descrita por primera vez en la literatura inglesa en 1948 por Ormond. Sin embargo, la primera descripción conocida de esta enfermedad fue realizada por el urólogo francés Albarran en 1905. Se caracteriza por una inflamación crónica e inespecífica del retroperitoneo, la cual puede atrapar y obstruir las estructuras que allí se encuentran, particularmente a los uréteres. Es frecuente que la enfermedad se detecte en etapas avanzadas, cuando se manifiesta por falla renal crónica. Los avances en las técnicas imaginológicas y el mayor conocimiento de la entidad han permitido que el diagnóstico se realice con mayor frecuencia en etapas más tempranas, cuando los pacientes aún no se encuentran obstruidos. Como resultado de las observaciones que muestran que los aneurismas inflamatorios de la aorta abdominal difieren de la enfermedad de Ormond sólo en el diámetro de la aorta inflamada, se ha sugerido que ambos síndromes representan variaciones de la misma patología, la cual se ha llamado “periaortitis crónica”.

Palabras clave:
fibrosis retroperitoneal idiopática
enfermedad de Ormond
obstrucción ureteral
periaortitis crónica.
Summary

Retroperitoneal fibrosis is an uncommon disease of unclear cause. It was described for the first time in English literature by Ormond, in 1948. However, the first known description of this disease was done by the French urologist Albarran, around 1905. It is characterized by chronic nonspecific inflammation of the retroperitoneum that can entrap and obstruct retroperitoneal structures, notably the urethers. This disease frequently is detected only after severe renal failure is present. However, advances in imaging techniques and increased awareness of the disease have resulted in an earlier diagnosis in unobstructed patients. As a result of the observations that showed that the only difference between an inflammatory aneurysm of the abdominal aorta and Ormond's disease is the diameter of the vessel, it had been suggested that both syndromes represent variations of the same pathology, which has been named “chronic periaortitis”

Key words:
idiopathic retroperitoneal fibrosis
Ormond's disease
Ureteral obstruction
chronic periaortitis
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