Learn About Retroperitoneal Fibrosis

What is the definition of Retroperitoneal Fibrosis?

Retroperitoneal fibrosis is a rare disorder that blocks the tubes (ureters) that carry urine from the kidneys to the bladder.

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What are the alternative names for Retroperitoneal Fibrosis?

Idiopathic retroperitoneal fibrosis; Ormond's disease

What are the causes of Retroperitoneal Fibrosis?

Retroperitoneal fibrosis occurs when extra fibrous tissue forms in the area behind the stomach and intestines. The tissue forms a mass (or masses) or tough fibrotic tissue. It can block the tubes that carry urine from the kidney to the bladder.

The cause of this problem is mostly unknown. It is most common in people aged 40 to 60. Men are twice as likely to develop the condition as women.

What are the symptoms of Retroperitoneal Fibrosis?

Early symptoms:

  • Dull pain in the abdomen that increases with time
  • Pain and change of color in the legs (due to decreased blood flow)
  • Swelling of one leg

Later symptoms:

  • Decreased urine output
  • No urine output (anuria)
  • Nausea, vomiting, changes in mental status caused by kidney failure and build-up of toxic chemicals in the blood
  • Severe abdominal pain with blood in the stool (due to death of intestinal tissue)
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What are the current treatments for Retroperitoneal Fibrosis?

Corticosteroids are tried first. Some health care providers also prescribe a drug called tamoxifen.

If corticosteroid treatment does not work, a biopsy should be done to confirm the diagnosis. Other medicines to suppress the immune system can be prescribed.

When medicine does not work, surgery and stents (draining tubes) are needed.

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What is the outlook (prognosis) for Retroperitoneal Fibrosis?

The outlook will depend on the extent of the problem and the amount of damage to the kidneys.

The kidney damage may be temporary or permanent.

What are the possible complications of Retroperitoneal Fibrosis?

The disorder may lead to:

  • Ongoing blockage of the tubes leading from the kidney on one or both sides
  • Chronic kidney failure
When should I contact a medical professional for Retroperitoneal Fibrosis?

Call your provider if you have lower abdomen or flank pain and less output of urine.

How do I prevent Retroperitoneal Fibrosis?

Try to avoid long-term use of medicines that contain methysergide. This drug has been shown to cause retroperitoneal fibrosis. Methysergide is sometimes used to treat migraine headaches.

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What are the latest Retroperitoneal Fibrosis Clinical Trials?
A Prospective Study to Evaluate the Efficacy and Safety of Cyclophosphamide in the Treatment of Idiopathic Retroperitoneal Fibrosis

Summary: This prospective, interventional, controlled study is aimed to evaluate the efficacy and safety of cyclophosphamide in the treatment of idiopathic retroperitoneal fibrosis, which refers to the chronic nonspecific inflammation of retroperitoneal fascia and adipose tissue that gradually evolves into fibroproliferative disease.

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A Prospective, Controlled, Interventional Study to Evaluate the Efficacy and Safety of Tocilizumab in the Treatment of Idiopathic Retroperitoneal Fibrosis

Methods: All the patients fulfilling diagnostic criteria of IRPF would be enrolled. The IRPF patients will accept Tocilizumab or Glucocorticoid monotherapy for 3 months. Endpoints: The primary endpoint is to investigate the response of Tocilizumab on IRPF patients; the secondary endpoints include the decrease of inflammatory markers, side effect.

What are the Latest Advances for Retroperitoneal Fibrosis?
Vascular thromboses with retroperitoneal fibrosis: a case report.
IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease.
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Who are the sources who wrote this article ?

Published Date: April 18, 2021
Published By: Kelly L. Stratton, MD, FACS, Associate Professor, Department of Urology, University of Oklahoma Health Sciences Center, Oklahoma City, OK. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Comperat E, Bonsib SM, Cheng L. Renal pelvis and ureter. In: Cheng L, MacLennan GT, Bostwick DG, eds. Urologic Surgical Pathology. 4th ed. Philadelphia, PA: Elsevier; 2020:chap 3.

Nakada SY, Best SL. Management of upper urinary tract obstruction. In: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, eds. Campbell-Walsh-Wein Urology. 12th ed. Philadelphia, PA: Elsevier; 2021:chap 89.

Privratsky AM, Barreto JC, Tumage RH. Abdominal wall, umbilicus, peritoneum, mesenteries, omentum, and retroperitoneum. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 21st ed. Philadelphia, PA: Elsevier; 2022:chap 44.

Shanmugam VK. Vasculitis and other uncommon arteriopathies. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 9th ed. Philadelphia, PA: Elsevier; 2019:chap 137.