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Genetic and Rare Diseases Information Center (GARD)

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Retroperitoneal fibrosis


Other Names for this Disease

  • Idiopathic retroperitoneal fibrosis
  • Ormond's disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is retroperitoneal fibrosis?

What are the symptoms of retroperitoneal fibrosis?

What causes retroperitoneal fibrosis?

How might retroperitoneal fibrosis be treated?

What is the typical prognosis for patients with retroperitoneal fibrosis?

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. The disorder may cause chronic unilateral obstructive uropathy or chronic bilateral obstructive uropathy.[1] Risk factors for retroperitoneal fibrosis include asbestos exposure, smoking, tumor, infection, trauma, radiotherapy, surgery, and use of certain drugs.[1][2][3]
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 many cases (idiopathic). Some cases occur in association with other factors, including:[1][2][3]

  • Asbestos exposure
  • Smoking
  • 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 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
  1. Vorvick LJ. Retroperitoneal fibrosis. MedlinePlus. March 17, 2011; http://www.nlm.nih.gov/medlineplus/ency/article/000463.htm. Accessed 10/15/2013.
  2. Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006; http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=DetailsSearch&Term=16427494%5Buid%5D. Accessed 10/15/2013.
  3. Goldoni M, Bonini S, Urban ML, Palmisano A, De Palma G, Galletti E, Coggiola M, Buzio C, Mutti A, Vaglio A. Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study. Ann Intern Med. 2014 Aug 5; 161(3):181-8. http://www.ncbi.nlm.nih.gov/pubmed/25089862. Accessed 10/15/2014.


Other Names for this Disease
  • Idiopathic retroperitoneal fibrosis
  • Ormond's disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.