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$Unique_ID{BRK03593}
$Pretitle{}
$Title{Cholangitis, Primary Sclerosing}
$Subject{Cholangitis, Primary Sclerosing Stenosing Cholangitis Fibrosing
Cholangitis Chronic Obliterative Cholangitis Primary Biliary Cirrhosis}
$Volume{}
$Log{}
Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc.
418:
Cholangitis, Primary Sclerosing
** IMPORTANT **
It is possible the main title of the article (Primary Sclerosing
Cholangitis) is not the name you expected. Please check the SYNONYMS listing
on the next page to find alternate names, disorder subdivisions, and related
disorders covered by this article.
Synonyms
Stenosing Cholangitis
Fibrosing Cholangitis
Chronic Obliterative Cholangitis
Information on the following disorder can be found in the Related
Disorders section of this report:
Primary Biliary Cirrhosis
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.
Primary Sclerosing Cholangitis is a rare collagen disorder involving
inflammation and blockage of the bile duct, liver ducts and gallbladder.
Episodes of pain and discomfort of the right upper section of the abdomen may
gradually become prolonged. This disorder predominately affects males.
Symptoms
The initial pain and discomfort of Primary Sclerosing Cholangitis affects the
upper right section of the abdomen and is associated with inflammation of the
common bile ducts. Episodes of pain gradually become prolonged. Loss of
appetite, nausea, or vomiting may occur, accompanied by weight loss. The
liver may become enlarged and noticeably sensitive. When collagen tissue
increases, an irregular pattern of thickening in the walls of the common bile
ducts blocks the transport of bile. When bile is blocked, it may be
absorbed into the blood in abnormal amounts causing a yellow discoloration of
the skin (jaundice) accompanied by chills, fever, or itching of the skin.
Fat soluble vitamins are poorly absorbed from the intestine. Blockages often
associated with inflammation or infection can also occur in the gall bladder
and the liver. Bacterial infections resulting from ulcerative colitis,
Crohn's Disease, retroperitoneal fibrosis, generalized vasculitis,
thyroiditis, or orbital pseudotumors may be associated with bile duct
blockages of Primary Sclerosing Cholangitis.
For more information, choose "ulcerative colitis" and "Crohn" as your
search terms in the Rare Disease Database.
Causes
The exact cause of Primary Sclerosing Cholangitis is not known. Researchers
believe an allergic or hypersensitivity reaction or a widespread disturbance
of collagen-containing tissues may be responsible for symptoms.
Affected Population
Primary Sclerosing Cholangitis is a rare disorder predominately affecting
middle-aged males, although it may affect males of any age.
Related Disorders
Symptoms of the following disorders can be similar to Primary Sclerosing
Cholangitis. Comparisons may be useful for a differential diagnosis:
Primary Biliary Cirrhosis, also known as Hanot's Cirrhosis, is a rare
liver condition occurring mainly in females. It is characterized by yellow
discoloration of the skin (jaundice) associated with obstruction and
inflammation of the large bile ducts which normally transport bile.
Additionally, abnormal cells accumulate in the liver associated with
degenerative liver disease (cirrhosis). Blockage may not occur in small bile
ducts. Some long-term severe cases of Primary Sclerosing Cholangitis may
develop into Primary Biliary Cirrhosis. (For more information on this
disorder, choose "Primary Biliary Cirrhosis" as your search term in the Rare
Disease Database.
Therapies: Standard
Treatment of Primary Sclerosing Cholangitis may benefit from imaging methods
such as X-ray or CAT Scan to identify the exact location and nature of
blockages. Surgery to remove blockages and enlarge narrowed bile ducts may
be of benefit to help prevent liver deterioration. Lost vitamins should be
replaced when required to prevent complications related to these
deficiencies. Antibiotics may be useful in controlling inflammation or
infection. The drug cholestyramine may be effective in controlling itching.
Other treatment is symptomatic and supportive.
Therapies: Investigational
Liver transplants for experimental treatment of the most severe cases of
Primary Sclerosing Cholangitis are being considered. Other treatments being
tested include drainage of blocked bile through tubes inserted in ducts and
enlarging abnormally narrowed bile ducts (at least temporarily) by inserting
a tiny balloon inside the duct and inflating it (endoscopic balloon
dilation). Effectiveness and side effects of these procedures and devices
have not been fully documented and more extensive research is being pursued
before their therapeutic value for Primary Sclerosing Cholangitis can be
evaluated.
This disease entry is based upon medical information available through
December 1988. 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 Primary Sclerosing Cholangitis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
National Digestive Diseases Information Clearinghouse
Box NDIC
Bethesda, MD 20892
(301) 468-2162
American Liver Foundation
998 Pomptom Avenue
Cedar Grove, NJ 07009
(201) 857-2626
(800) 223-0179
The United Liver Foundation
11646 West Pico Blvd.
Los Angeles, CA 90064
(213) 445-4204 or 445-4200
Children's Liver Foundation
14245 Ventura Blvd.
Sherman Oaks, CA 91423
(818) 906-3021
References
PRIMARY SCLEROSING CHOLANGITIS: J.J. Alberti-Flor, et al.; South Med J
(February 1985, issue 78(2)). Pp. 173-177.
DIAGNOSIS AND TREATMENT OF PRIMARY SCLEROSING CHOLANGITIS: R.H.
Wiesner, et al.; Semin Liver Dis (August 1985, issue 5(3)). Pp. 241-253.
THERAPEUTIC INVESTIGATIONS IN PRIMARY SCLEROSING CHOLANGITIS: R. Grijm,
et al.; Dig Dis Sci (August 1986, issue 31(8)). Pp. 792-798.