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retroperitoneal fibrosis (Ormond disease)
Etiology:
- IgG4 disease
- idiopathic
Epidemiology:
- rare
Pathology:
- fibrosis in the retoperitoneum
Clinical manifestations:
- dull pain in the abdomen that increases with time
- unilateral leg edema
- oliguria may progress to anuria
Laboratory:
- renal function tests
Special laboratory:
- renal ultrasound
- tissue biopsy
Radiology:
- computed tomography of the abdomen may reveal a retroperitoneal mass
- magnetic resonance imaging (MRI) of the abdomen
Complications:
- obstructive uropathy may result from pressure on the ureters
Management:
- systemic glucocorticoids are 1st line
- tamoxifen has been used
- surgery & stents (if needed)
Related
retroperitoneum (peri/pararenal fat)
General
fibrosis
References
- PubMed Health: Retroperitoneal fibrosis
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001497/
- Biyani CS
eMedicine: Retroperitoneal Fibrosis
http://emedicine.medscape.com/article/458501-overview