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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Retroperitoneal fibrosis


Other Names for this Disease
  • Idiopathic retroperitoneal fibrosis
  • Ormond's disease
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Your Question

I have been diagnosed with retroperitoneal fibrosis. Can you provide me with information about this disease?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is retroperitoneal fibrosis?

Retroperitoneal fibrosis is a slowly progressive disorder in which the tubes that carry urine from the kidneys to the bladder (ureters) and other abdominal organs are blocked by a fibrous mass and inflammation in the back of the abdomen. Doctors don't know why these masses form. The disorder may cause chronic unilateral obstructive uropathy or chronic bilateral obstructive uropathy.[1]
Last updated: 10/15/2013

What are the symptoms of retroperitoneal fibrosis?

Early symptoms of retroperitoneal fibrosis may include:[1]

  • Dull pain in the abdomen that increases with time
  • Swelling of one leg
  • Decreased circulation in the legs leading to pain and discoloration
  • Severe abdominal pain with hemorrhage due to ischemic bowel

Late symptoms of retroperitoneal fibrosis may include:[1]

  • Decreased urine output
  • Total lack of urine (anuria)
  • Nausea, vomiting, changes in thinking caused by kidney failure and the resulting build-up of toxic chemicals in the blood.
Last updated: 10/15/2013

What causes retroperitoneal fibrosis?

The cause of retroperitoneal fibrosis is unknown in 2/3 of cases (idiopathic); the rest are secondary to other factors, including:[1][2]

  • Neoplasms (tumor)
  • Infections
  • Trauma
  • Radiotherapy
  • Surgery
  • Use of certain drugs
Last updated: 10/15/2013

How might retroperitoneal fibrosis be treated?

Treatment of retroperitoneal fibrosis may include:[1][2]

Surgery to remove the mass and free the ureters may be required. In some cases, the ureters will be moved to a different position in the body or wrapped in fat tissue harvested from other areas to prevent recurrence of the fibrosis. Stents (drainage tubes) placed in the ureter or in the renal pelvis may provide short-term relief of the symptoms until the mass can be removed. Corticosteroid therapy (a type of anti-inflammatory medicine) may help if surgery can't be done due to other medical conditions. In addition, some doctors use the drug tamoxifen to treat this condition.[2]

Last updated: 10/15/2013

What is the goal of treatment for retroperitoneal fibrosis ?

The aims of treatment of idiopathic retroperitoneal fibrosis are multiple:[2]

  • To stop the progression of the fibro-inflammatory reaction
  • To inhibit or relieve the obstruction of the ureters or other retroperitoneal structures 
  • To switch off the acute-phase reaction (symptoms can include side, back, or abdominal pain) and its systemic manifestations (such as fatigue, low-grade fever, nausea, weight loss, and myalagias)
  • To prevent disease recurrence or relapse
Last updated: 10/15/2013

What is the typical prognosis for patients with retroperitoneal fibrosis?

The outlook for patients with retroperitoneal fibrosis is usually considered to be good, but severe complications such as chronic renal failure, requiring kidney transplant, can arise.[1][2]
Last updated: 10/15/2013

References