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$Unique_ID{BRK03607}
$Pretitle{}
$Title{Cirrhosis, Primary Biliary}
$Subject{Cirrhosis, Primary Biliary Hanot's Cirrhosis PBC Wilson's Disease
Extrahepatic Bile Duct Obstruction Obstructive Biliary Cirrhosis Alcoholic
Cirrhosis}
$Volume{}
$Log{}
Copyright (C) 1989, 1990, 1991 National Organization for Rare Disorders,
Inc.
602:
Cirrhosis, Primary Biliary
** IMPORTANT **
It is possible that the main title of the article (Primary Biliary
Cirrhosis) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
Hanot's Cirrhosis
PBC
Information on the following diseases can be found in the Related
Disorders section of this report:
Wilson's Disease
Extrahepatic Bile Duct Obstruction
Obstructive Biliary Cirrhosis
Alcoholic 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 Biliary Cirrhosis is a chronic progressive liver disorder thought
to be related to abnormalities in the immune system. Obstruction of the
small bile ducts is accompanied by yellow discoloration of the skin
(jaundice). Excessive amounts of copper accumulate in the liver, and fibrous
or granular hardening (induration) of the soft liver tissue develops. This
disorder occurs mainly in women during the fourth to the seventh decade of
life, and is marked by four progressive stages.
Symptoms
Primary Biliary Cirrhosis is characterized initially by blockage, then
deterioration and loss of the small bile ducts inside the liver. This
results in the abnormal retention (cholestasis) of bile acids, bilirubin,
copper, and other substances that are normally excreted into bile. These
substances cause further damage to liver cells. Itching often results from
the abnormal passage of these substances into the blood, skin and other soft
tissues of the body. Excessive amounts of fat soluble substances (lipids)
and cholesterol are found circulating in the blood. Yellow nodules or
plaques form on the skin and possibly in internal organs.
The bile acid concentration in the intestines is inadequate for complete
digestion and absorption of fats (triglycerides) in the diet. Additionally,
normal absorption of Vitamins A, D, E, and K as well as calcium may be
diminished, and iron deficiency anemia may develop. Loss of bone density
(osteoporosis) and bone softening (osteomalacia) occurs in approximately
twenty-five percent of patients.
Primary Biliary Cirrhosis is divided into four symptomatic stages. In
stage I, the liver is enlarged and destructive lesions affect the large bile
ducts. Unexplained itching is common and is often worse at night. Increased
amounts of melanin pigmentation appear in the skin, and patches of skin may
be shed (excoriation). Fatigue and unexplained weight loss are also common.
In stage II, lesions are more widespread but less specific. Normal bile
ducts may be lost and increased numbers of poorly formed bile ducts with
irregularly shaped interior spaces (lumens) are seen on biopsy tests.
Fibrous cells infiltrate and spread within the liver. Stoppage of bile is
limited to portal areas.
Stage III is characterized by more progressive lesions in the liver, with
fibrous infiltration becoming very widespread. Nodule formation may now
become apparent. Intestinal malabsorption of nutrients may occur in some
patients, resulting in nocturnal diarrhea, frothy, bulky stools, or weight
loss even though the patient has a voracious appetite and increased caloric
intake. Yellow plaques (xanthomas) appear on the skin in approximately ten
percent of patients. These may be found on the palms of the hands, soles of
the feet, elbows, knees, in tendons of wrists and/or ankles, and on the
buttocks. Bone pain is common, as are spontaneous collapse of spinal bone
segments and hairline fractures of the ribs, usually due to loss of bone
density (osteoporosis).
Stage IV represents the end stage of lesion formation, with widespread
hardening of soft liver tissue (cirrhosis) and regenerative nodules.
Yellowish discoloration (jaundice) of the skin becomes pronounced due to the
presence of excess bilirubin. It is difficult to distinguish stage IV from
other types of cirrhosis.
Causes
The exact cause of Primary Biliary Cirrhosis is not known. Some scientists
believe an autoimmune abnormality may be the cause. Autoimmune disorders are
caused when the body's natural defenses (antibodies or lymphocytes) against
invading organisms suddenly begin to attack healthy tissue.
Affected Population
Primary Biliary Cirrhosis affects females in approximately ninety percent of
cases. Symptoms usually appear in the fourth to seventh decade of life.
Related Disorders
Symptoms of the following disorders can be similar to those of Primary
Biliary Cirrhosis. Comparisons may be useful for a differential diagnosis:
Wilson's Disease is a rare genetic disorder of copper metabolism
characterized by excess storage of copper in the body tissues, particularly
in the liver, brain and corneas of the eyes. It leads eventually to liver
disease, brain dysfunction, and a characteristic rusty-brown colored ring
around the cornea of each eye known as a Kayser-Fleischer ring. (For more
information on this disorder, choose "Wilson" as your search term in the Rare
Disease Database).
Extrahepatic Bile Duct Obstruction originates outside the liver but may
produce symptoms similar to those of Primary Biliary Cirrhosis.
Obstructive Biliary Cirrhosis is characterized by fibroid or granular
hardening of the soft liver tissue due to bile duct obstruction rather than
deterioration inside the liver typical of Primary Biliary Cirrhosis.
Alcoholic Cirrhosis is characterized by gradual hardening of the soft
tissue of the liver (cirrhosis) that frequently develops in chronic
alcoholism. The early stage is marked by liver enlargement due to fatty
infiltration with mild fibrosis. In late stages, normal liver lobes are
replaced with small nodules, separated by a framework of fine fibrous tissue
strands (hobnail liver).
Therapies: Standard
Treatment of Primary Biliary Cirrhosis is often directed at symptoms. The
drug cholestyramine relieves itching in almost all patients. Colestipol
hydrochloride may also be effective against itching. Large-volume
plasmapheresis may relieve itching in patients who do not respond to drug
treatment. This procedure is a method for removing unwanted substances
(toxins, metabolic substances and plasma parts) from the blood. Blood is
removed from the patient and blood cells are separated from plasma. The
patient's plasma is then replaced with other human plasma and the blood is
retransfused into the patient.
Malabsorption of fat-soluble vitamins may be treated with vitamin K 1, A,
D, and calcium supplementation. Iron deficiency anemia responds to oral iron
supplements. Additional folic acid is recommended for patients taking
cholestyramine because this drug can cause a folic acid deficiency. Folic
acid and cholestyramine should be taken several hours apart since they may
react and prevent folic acid absorption when taken together. Loss of fat in
the stools (steatorrhea) may be treated by a low-fat diet supplemented with
medium-chain triglycerides to maintain high caloric intake.
Therapies: Investigational
Experimental treatment of Primary Biliary Cirrhosis may involve liver
transplantation. More research is necessary before this procedure can be
recommended for all but the most severe cases of this disorder. Trials of
several drugs including colchicine, prednisolone, D-penicillamine,
azathioprine, cyclosporin A and chlorambucil are being conducted. However,
long-term safety and effectiveness of these drugs has not been determined.
The FDA has approved for testing the orphan drug Actigall
(Ursodeoxycholic Acid) for treatment of Primary Biliary Cirrhosis. The drug
is manufactured by the Ciba-Geigy Corp., 556 Morris Ave., Summit, NJ, 07901.
The orphan drug Urosofalk (Ursodeoxycholic Acid) is being tested by the
FDA as a treatment of Primary Biliary Cirrhosis. The drug is manufactured by
Interfolk U.S., Inc., 25 Margaret St., Plattsburgh, NY, 12901.
This disease entry is based upon medical information available through
July 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 Primary Biliary Cirrhosis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Primary Billary Cirrhosis Patient Support Network
Box 177
Tamworth, Ontario KOK 3GO
Canada
(613) 379-2534
American Liver Foundation
1425 Pompton Ave.
Cedar Grove, N.J. 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
National Digestive Diseases Information Clearinghouse
Box NDDIC
Bethesda, MD 20892
(301) 468-6344
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 225-226.
TRANSPLANTATION OF LIVER, HEART, AND LUNGS FOR PRIMARY BILIARY CIRRHOSIS
AND PRIMARY PULMONARY HYPERTENSION: J. Wallwork, et al.; Lancet (July 25,
1987, issue 2(8552)). Pp. 182-185.
CLINICAL AND STATISTICAL ANALYSES OF NEW AND EVOLVING THERAPIES FOR
PRIMARY BILIARY CIRRHOSIS: R.H. Wiesner, et al.; Hepatology (May-June 1988,
issue 8(3)). Pp. 668-676.
TREATMENT OF PRURITIS IN PRIMARY BILIARY CIRRHOSIS WITH RIFAMPIN. RESULTS
OF A DOUBLE-BLIND, CROSSOVER, RANDOMIZED TRIAL: C.N. Ghent, et al.;
Gastroenterology (February 1988, issue 94(2)). Pp. 488-493.