Learn About Retroperitoneal Fibrosis
Retroperitoneal fibrosis is a rare disorder that blocks the tubes (ureters) that carry urine from the kidneys to the bladder.
Idiopathic retroperitoneal fibrosis; Ormond's disease
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.
Early symptoms:
- Dull pain in the abdomen or flank 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)
Corticosteroids are tried first. Some health care providers also prescribe a medicine 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.
Guy's And St. Thomas Hospitals
Tim O'brien practices in London, United Kingdom. Mr. O'brien is rated as an Elite expert by MediFind in the treatment of Retroperitoneal Fibrosis. His top areas of expertise are Retroperitoneal Fibrosis, Renal Oncocytoma, Renal Cell Carcinoma (RCC), Nephrectomy, and Endoscopy.
Aurora Urology
Alex Zacharias is an Urologist in Milwaukee, Wisconsin. Dr. Zacharias is rated as a Distinguished provider by MediFind in the treatment of Retroperitoneal Fibrosis. His top areas of expertise are Prostate Cancer, Familial Prostate Cancer, Muscle Invasive Bladder Cancer, Reconstructive Urology Surgery, and Ureteroscopy.
Augusto Vaglio practices in Florence, Italy. Mr. Vaglio is rated as an Elite expert by MediFind in the treatment of Retroperitoneal Fibrosis. His top areas of expertise are Retroperitoneal Fibrosis, Granulomatosis with Polyangiitis, Vasculitis, Kidney Transplant, and Tissue Biopsy.
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.
The disorder may lead to:
- Ongoing blockage of the tubes leading from the kidney on one or both sides
- Chronic kidney disease or failure
Contact your provider if you have lower abdomen or flank pain and less output of urine.
Try to avoid long-term use of medicines that contain methysergide, ergot alkaloids, or dopamine agonists. These medicines have been shown to rarely cause retroperitoneal fibrosis.
Summary: Retroperitoneal fibrosis refers to a group of diseases characterized by hyperplasia of the fibrosclerotic tissues in the retroperitoneal space, which can compress the surrounding ureters and inferior vena cava and cause serious complications such as aortic aneurysm, renal failure, and even death. The lesion is diffuse and difficult to resect. corticosteroid is the first-line medication, but the re...
Summary: Adult patients with a diagnosis of idiopathic retroperitoneal fibrosis Prospective multicentric cohort study Intervention : administration of prednisone during 9 to 21 months at 1mg/kg/day at inclusion.
Published Date: April 01, 2025
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 C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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. St Louis, MO: Elsevier; 2022:chap 44.
Shanmugam VK. Vasculitis and other uncommon arteriopathies. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 10th ed. Philadelphia, PA: Elsevier; 2023:chap 138.
Varga J. Systemic sclerosis (scleroderma). In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 246.