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Morbus Ormond (idiopatic retroperitoneal fibrosis)


Authors: A. Michaligová 1;  L. Plank 2;  A. Ježíková 1;  V. Maňka 1;  P. Makovický 1;  M. Mokáň 1
Authors‘ workplace: I. Interná klinika Jesseniovej lekárskej fakulty UK a Univerzitnej nemocnice Martin, Slovenská republika, prednosta prof. MUDr. Marián Mokáň, DrSc., FRCP Edin 1;  Ústav patologickej anatómie Jesseniovej lekárskej fakulty UK a Univerzitnej nemocnice Martin, Slovenská republika, prednosta prof. MUDr. Lukáš Plank, CSc. 2
Published in: Vnitř Lék 2011; 57(5): 511-515
Category: Case Reports

Overview

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of fibrotic tissue around the abdominal aorta and iliac arteries and often involves structures as ureters and the inferior vena cava. The age at onset of signs and symptoms is between 40–60 years, males predominane over females. In most cases the clinical manifestation is presented as compressive syndrom of ureters, therefore the first known cases were described by urologists. In this report we present the case of 37-years old male examinated for persistent fever about 38 °C and high inflammatory activity in spite of empiric antibiotic therapy. Positron emission to­mography (PET) showed locality of high metabolic activity of fluorodeoxyglucose with maximum paraaortal left. Microscopic examination of extracted mass showed presence of fibrous and inflammatory components. With clinical presentation, imaging and histological findings we made out the diagnosis of idiopathic retroperitoneal fibrosis – morbus Ormond.

Key words:
idiopatic retroperitoneal fibrosis – Ormond’s disease – fever


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