[Ormond's disease: Experience with five cases]

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: 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.