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

Diagnostic procedures

• 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)

More general terms

• fibrosis

Additional terms

• retroperitoneum (peri/pararenal fat)

Patient information

retroperitoneal fibrosis patient information