RESUMEN
La enfermedad de Ormond (EO) es una enfermedad infrecuente con una incidencia aproximada de 1/1.000.000 personas-año. La etiología en la mayoría de los casos es desconocida, y en la EO secundaria se han implicado múltiples procesos patogénicos. La EO se caracteriza por la presencia de una masa fibroinflamatoria retroperitoneal con tres formas clínicas diferentes: i) fibrosis retroperitoneal; ii) fibrosis perianeurismática retroperitoneal, y iii) inflamación de los aneurismas de la aorta abdominal. El manejo clásico se basa en el tratamiento quirúrgico y puede asociarse a esteroides. En estos últimos años se han empleado otros inmunosupresores sin resultados bien establecidos. Presentamos cinco casos acontecidos en el Hospital Clínico Universitario de Salamanca durante el período 20002008. Destacamos la falta de estudios para establecer guías de práctica clínica que faciliten el manejo y mejoren el pronóstico (AU)
Ormond´s disease (OD) is an uncommon process with an annual incidence nearing 1 per million inhabitants. The etiology in most of the cases is unknown and several pathogenic mechanisms are implicated in secondary OD. Ormond disease is characterized by a fibrotic and inflammatory mass with three different clinical features: i) retroperitoneal fibrosis, ii) perianeurysmatic retroperitoneal fibrosis and iii) inflammatory abdominal aortic aneurysms. Classic management is based on surgical treatment associated or not with steroids. Immunosuppressive agents have been used in the last years with unclear results. We report five cases from the University Hospital of Salamanca occurring during 20002008. We highlight the lack of trials designed to establish clinical guidelines for the treatment of the disease and improvement of outcome (AU)
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Fibrosis Retroperitoneal/diagnóstico , Fibrosis Retroperitoneal/cirugía , Nefrostomía Percutánea , Factores de Riesgo , Inmunohistoquímica/métodos , Inmunohistoquímica/tendencias , Fibrosis Retroperitoneal/fisiopatología , Estudios Retrospectivos , Tamoxifeno/uso terapéutico , Prednisona/uso terapéutico , Imagen por Resonancia Magnética/métodos , Azatioprina/uso terapéutico , Corticoesteroides/uso terapéutico , Inmunosupresores/uso terapéuticoRESUMEN
Ormond's disease (OD) is an uncommon process with an annual incidence nearing 1 per million inhabitants. The etiology in most of the cases is unknown and several pathogenic mechanisms are implicated in secondary OD. Ormond disease is characterized by a fibrotic and inflammatory mass with three different clinical features: i) retroperitoneal fibrosis, ii) perianeurysmatic retroperitoneal fibrosis and iii) inflammatory abdominal aortic aneurysms. Classic management is based on surgical treatment associated or not with steroids. Immunosuppressive agents have been used in the last years with unclear results. We report five cases from the University Hospital of Salamanca occurring during 2000-2008. We highlight the lack of trials designed to establish clinical guidelines for the treatment of the disease and improvement of outcome.
