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Vol. 6. Issue 4.
Pages 199-202 (July - August 2010)
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Vol. 6. Issue 4.
Pages 199-202 (July - August 2010)
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Ormond's disease: Experience with five cases
Enfermedad de Ormond: experiencia de cinco casos
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Natalia Castro-Iglesiasa, Moncef Belhassen-Garcíaa,
Corresponding author
mbelhassen@hotmail.com

Corresponding author.
, Virginia Velasco-Tiradoa, Adela Carpio-Péreza, Sandra Inés-Revueltab, Sheila Martín-Barbaa, Javier Pardo-Lledíasc
a Servicio de Medicina Interna III, Hospital Clínico Universitario de Salamanca, Salamanca, Spain
b Servicio de Medicina Interna II, Hospital Clínico Universitario de Salamanca, Salamanca, Spain
c Servicio de Medicina Interna, Hospital General de Segovia, Segovia, Spain
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Abstract

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: 1) retroperitoneal fibrosis, 2) perianeurysmatic retroperitoneal fibrosis and 3) 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.

Keywords:
Ormond's disease
Retroperitoneal fibrosis
Chronic periaortitis
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 2000–2008. Destacamos la falta de estudios para establecer guías de práctica clínica que faciliten el manejo y mejoren el pronóstico.

Palabras clave:
Enfermedad de Ormond
Fibrosis retroperitoneal
Periaortitis crónica
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