Vnitr Lek 2008, 54(3):282-286

Idiopathic retroperitoneal fibrosis - Ormond's disease

M. Průcha^1,*, P. Beňo², M. Bartůněk², J. Votrubová³, L. Sedláčková¹, R. Kříž⁴

¹ Oddělení klinické biochemie hematologie a imunologie Nemocnice Na Homolce, Praha, prim. MUDr. Miroslav Průcha, Ph.D.

² Chirurgické oddělení Nemocnice Na Homolce, Praha, prim. MUDr. Pavel Beňo

³ Oddělení nukleární medicíny Nemocnice Na Homolce, Praha, doc. MUDr. Otakar Bělohlávek, CSc.

⁴ Radiodiagnostické oddělení Nemocnice Na Homolce, Praha, prim. MUDr. Vladimíra Janoušková, CSc.

Ormond disease - idiopathic retroperitoneal fibrosis - is a rare condition characterized in situ by the development of fibrous plaques in the retroperitoneal space and anatomicaly dependent structures. The associated encasement of both ureters and progress to hydronefrosis of the kidney are typical clinical manifestations. Less typical manifestations are possible (for example chronic periaortitis), where clinical diagnosis is more difficult. The laboratory findings are not specific for this disease and a biopsy is not always possible for anatomical reasons. In these cases, the use of positron emission tomography/computed tomography - has been found to be the solution, specifically for patients with periaortitis. Ormond disease is generally idiopathic, and secondary - to the use of certain drugs, malignant diseases, infections. Idiopathic retroperitoneal disease is thought to result from the clinical manifestation of a systemic autoimmune disease. The purpose of this article is to present two casuistics, one of a less than usual clinical manifestation. Both positron emission tomography/computed tomography were used in the diagnostics. The treatment of Ormond disease involves the combination of surgical and immunosuppressive treatment.

Keywords: Ormond disease; idiopathic retroperitoneal fibrosis; diagnostics; therapy

Received: November 12, 2007; Accepted: December 31, 2007; Published: March 1, 2008  Show citation