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$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.