$Unique_ID{BRK04184} $Pretitle{} $Title{Retroperitoneal Fibrosis} $Subject{Retroperitoneal Fibrosis Idiopathic Retroperitoneal Fibrosis Ormonds Disease Carcinoid Syndrome Scleroderma Vasculitis } $Volume{} $Log{} Copyright (C) 1991 National Organization for Rare Disorders, Inc. 849: Retroperitoneal Fibrosis ** IMPORTANT ** It is possible that the main title of the article Retroperitoneal Fibrosis) is not the name you expected. Please check the Synonym listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Idiopathic Retroperitoneal Fibrosis Ormonds Disease Information on the following diseases can be found in the Related Disorders section of this report: Carcinoid Syndrome Scleroderma Vasculitis General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Retroperitoneal Fibrosis is a rare disorder in which there is a formation of fiber-like tissue behind the membrane that lines the cavity of the abdomen (peritoneum). This abnormal tissue growth usually affects the tubes that carry the urine from the kidney to the bladder (ureters). Often these tubes are blocked by the excess tissue. In most cases the cause of this disorder is unknown. Symptoms The most common symptom of Retroperitoneal Fibrosis is pain in the lower back or abdomen. In many cases this pain is vague and difficult to localize. Other symptoms may be weight loss, fever, nausea, a low level of red blood cells (anemia), and loss of appetite. Impaired movement of a limb may occur intermittently and yellow pigmentation of the skin (jaundice) may be present. Occasionally there may be bleeding in the stomach and intestine. In about ten percent of the cases there may be difficulty urinating. Upon examination by a physician, a mass can be felt in the rectum or abdomen in about fifteen percent of patients with this disorder. In some patients the largest vein in the body that returns blood to the heart (inferior vena cava) may be encased by the fibrous tissue. This encasement rarely causes obstruction of the vein. Complications of Retroperitoneal Fibrosis may be high blood pressure (hypertension) as well as blood vessel blockage. In some rare cases Retroperitoneal Fibrosis may become malignant. Causes The exact cause of Retroperitoneal Fibrosis is not known in about two-thirds of the patients. A drug used in the treatment and prevention of migrane headaches (methysergide) may be the cause of this rare disorder in twelve percent of the cases. Malignant tumors are associated with Retroperitoneal Fibrosis in eight percent of the patients. Tissue that has been injured by trauma or surgery may be a factor in some cases. Affected Population Retroperitoneal Fibrosis affects males twice as often as females. Seventy percent of the patients with this disorder are in their fifth to seventh decade of life. Retroperitoneal Fibrosis can affect children but it is very rare. Related Disorders Symptoms of the following disorders can be similar to those of Retroperitoneal Fibrosis. Comparisons may be useful for a differential diagnosis: Carcinoid Syndrome is a rare, malignant disorder that affects the small bowel, pancreas, and/or stomach. Slow growing tumors can spread to the lungs, liver and ovary. Symptoms of this disorder may include flushing, diarrhea, wheezing, stomach pain, and blockage of arteries. (For more information on this disorder, choose "Carcinoid " as your search term in the Rare Disease Database). Scleroderma is a group of chronic disorders characterized by fiber-like tissue growth (fibrosis), degenerative changes, and vascular abnormalities of the skin. Scleroderma is the hardening and shrinking of the connective tissues of any part of the body. (For more information on this disorder, choose "Scleroderma" as your search term in the Rare Disease Database). Vasculitis is an inflammation of the blood vessel system which includes the veins, arteries and capillaries. This disorder may occur alone or in conjunction with allergic and rheumatic diseases. Symptoms of this disorder may be formation of blood clots, weakening of vessel walls, muscle pain, joint pain, fever, weight loss, loss of appetite, abdominal pain and shortness of breath. (For more information on this disorder, choose "Vasculitis" as your search term in the Rare Disease Database). Therapies: Standard Treatment of Retroperitoneal Fibrosis depends on the location and extent of the tissue growth. Surgery is often very successful in freeing an organ that has been constricted by Retroperitoneal Fibrosis. Steroid drug therapy may be used along with surgery, or in patients who are at high risk if surgery is performed. In other cases this disorder can subside on it's own with no treatment needed. Therapies: Investigational Retroperitoneal Fibrosis has been treated successfully with the drug Azathioprine in a few cases. Another drug, Progesterone, has been used in Latin America for the treatment of Retroperitoneal Fibrosis. More research is needed to determine the safety and effectiveness of these experimental treatments. This disease entry is based upon medical information available through June 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Retroperitoneal Fibrosis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 203-746-6518 National Kidney Foundation 2 Park Avenue New York, NY 10016 212-889-2210 National Digestive Diseases Information Clearinghouse P.O. Box NDDIC Bethesda, MD 20892 301-468-6344 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, Editor-In-Chief; Little, Brown and Co., 1987. Pp. 455-56. CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. P. 273. IDIOPATHIC RETROPERITONEAL FIBROSIS--IS SERUM ALKALINE PHOSPHATASE A MARKER OF DISEASE ACTIVITY: I.G. Barrison, et al.: Postgrad Med J; (Mar 1988, issue 64(749)). Pp. 239-41. NON-OPERATIVE MANAGEMENT OF RETROPERITONEAL FIBROSIS: P.M. Higgins, et al.: Br J Surgery; (June 1988, issue 75(6)). Pp. 573-7. IDIOPATHIC RETROPERITONEAL FIBROSIS--AN UPDATE: P.M. Higgins, et al.: Dig Dis; (1990, issue 8(4)). Pp. 206-22.