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What is Retroperitoneal fibrosis

Retroperitoneal fibrosis is a rare autoimmune related disorder that blocks the tubes (ureters) that carry urine from the kidneys to the bladder. Retroperitoneal fibrosis occurs when extra fibrous tissue forms in the area behind the stomach and intestines. The tissue forms a mass (or masses) that can block the tubes that carry urine from the kidney to the bladder. The cause of this problem is not known. It is most common in people aged 40 – 60. Men are twice as likely to develop the condition as women. Early symptoms include: dull pain in the abdomen that increases with time, pain and change of color in the legs (due to decreased blood flow), and swelling of one leg. Later symptoms include: decreased urine output, no urine output (anuria), nausea, vomiting, changes in thinking caused by kidney failure and build-up of toxic chemicals in the blood, and severe abdominal pain with hemorrhaging (due to death of intestinal tissue). 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.

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 or vessels become blocked by a fibrous mass and inflammation in the back of the abdomen. The disorder may cause pain in the abdomen that worsens with time, pain or swelling of the legs, decreased urine output, and swelling of the scrotum in men.[1][2] Risk factors for retroperitoneal fibrosis include asbestos exposure, smoking, tumor, infection, trauma, radiotherapy, surgery, and use of certain drugs.[1][2][3][4]Treatment may include corticosteroids, tamoxifen, stents or surgery.[1]

Retroperitoneal fibrosis is characterized by inflammation and excessive scar tissue that develops in the back of the abdominal cavity. It can occur at any age, but appears most often between the ages of 40 and 60.[1][2] Men are twice as likely to develop the condition as women.[1]

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
https://rarediseases.info.nih.gov/diseases/9568/retroperitoneal-fibrosis

Related Autoimmune Patient Groups

Scleroderma Foundation
Scleroderma Foundation
Vasculitis Foundation
Vasculitis Foundation
National Kidney Foundation
National Kidney Foundation

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