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Clin Rheumatol. 2013 Mar;32 Suppl 1:S67-70. Epub 2010 Apr 15.

Thrombosis of the inferior vena cava related to Ormond's disease.

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  • 1Division of Angiology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036 Graz, Austria.


A 46-year-old female patient presenting with unspecific lower back pain, diffuse abdominal pain, and slightly elevated body temperature was referred to the hospital. The X-ray of the spine detected an unspecific sclerotic lesion of the third lumbar vertebra; an abdominal computed tomography (CT) scan was performed. In this scan, a sheeting of the infrarenal abdominal aorta, a streaky densification of the periaortal fatty tissue, and a nearly complete thrombotic occlusion of the inferior vena cava and both iliac veins was detected. As these findings are typical for acute aortitis and this is a life-threatening disease, the patient was transferred to a university hospital. Imaging work-up including ultrasound, magnetic resonance imaging, and 18-flourodeoxyglucose positron emission tomography were performed after transfer. These examinations showed acute aortitis due to Ormond's disease. Furthermore, there was a thrombotic occlusion of the inferior vena cava due to a compression of the vein by inflamed connective tissue. This is a rare finding in Ormond's disease. We decided to treat our patient with a combined therapy including glucocorticoids and tamoxifen for 2 years. During these 2 years and the further follow-up period, the patient showed no relapse of the inflammatory disease. Retroperitoneal fibrosis is a rare but increasingly recognized disease characterized by the presence of fibro-inflammatory tissue, usually surrounding the abdominal aorta and the iliac arteries. This fibrotic tissue extends into the retroperitoneum and thus encases structures, for example, the ureters. Retroperitoneal fibrosis is generally idiopathic. It can also be secondary to certain drugs, malignant diseases, infections, surgery, and other triggering factors. Ormond's disease may lead to serious complications like renal failure. This can be avoided by successful treatment when diagnosed early.

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