What is Primary Biliary Cirrhosis?
Primary Biliary Cirrhosis (PBC) is a chronic liver disease that slowly destroys the bile ducts within the liver (intrahepatic bile ducts). The name is somewhat deceptive since cirrhosis only occurs in the last stage of the disease after many years of inflammation. With early diagnosis and proper medications, most will never reach the cirrhosis stage of PBC.
PBC is also called
"Chronic Nonsuppurative Destructive Cholangitis"
and "Primary Autoimmune Cholangitis." However,
these alternative terms are not widely accepted and therefore not in use.
The cause of PBC is still
unknown, but it is not alcohol or drug induced or contagious. Current studies
suggest it may involve autoimmunity, infection, or genetic predisposition, and
does seem to appear more often in certain families. Women are affected 10 times
more than men, and PBC is usually diagnosed in patients between the ages of 35
to 60 years.
Upon
diagnosis, some doctors may suggest their patient
- Start a reduced sodium
diet and or low fat diet.
- Lower red meat intake and avoid raw meats, fish
& shellfish.
- Drink plenty of water and other fluids such as juice.
- Take Calcium and Vitamin D supplements. Calcium is the most common mineral in the body and is required for proper functioning of most organs. It is particularly needed in the normal development of the bones and teeth. Osteoporosis is a bone disease where calcium leaves the bones, causing them to weaken, and is commonly associated with PBC.
- Avoid or lower intake of alcohol
- Lower caffeine intake
- Avoid undue stress
- Exercise, if possible.
Walking is the most common recommendation for exercise.
- Eat smaller meals four or five times a day.
- Stop smoking
The above suggestions have been found to be very helpful in liver disease, but are common sense in healthy living.
The number of patients being
diagnosed at the asymptomatic stage has risen dramatically over the past few
years due to widespread laboratory screening. Typically, the blood lab pattern
reveals an elevated alkaline phosphatase level with a
normal bilirubin. Bilirubin
does not increase until final disease stage.
There are four disease stages of PBC.
Medical tests used to confirm PBC:
- A liver biopsy helps confirm the diagnosis and stage of the disease.
- Blood lab tests
that show liver dysfunction:
- Liver function tests
- Antimitochondrial antibodies. Positive AMA is found in about 95% of PBC patients. (Biliary enzymes & Pathology-PBC disease progression slides)
- Serum cholesterol and
lipoproteins may be increased.
- Haptoglobin & ACE
levels may be altered
- Diagnostic tests
- Other exams and tests
The disease advances slowly over a period of years. Most patients lead normal lives for years without symptoms, depending on how early diagnosis is made. There is no cure for PBC, but patients are showing good results in slowing the disease progress with URSO 250, Actigall and methotrexate. With the current medications, it is becoming more common for the PBC patient to live a long life without any complications from PBC, and in some cases actually lowering the disease stage from original diagnosis stage.
PBC is considered an autoimmune disease. When diagnosed, it may be associated with one or more other autoimmune diseases such as rheumatoid arthritis, sjogrens syndrome, raynauds phenomenon, celiac sprue, systemic lupus erythematosis ( SLE or lupus) or scleroderma.
Patients remain without symptoms for many years, and some may never notice symptoms other than itching and fatigue. The initial symptoms vary among PBC patients, and because of the varying symptoms can sometimes make it difficult for doctors to actually diagnose PBC. The varying symptoms may be due to the individual, autoimmune nature or other diseases associated with PBC. Symptoms may be present in any combination and include any of the following:
- Chronic fatigue may be the first symptom the patient notices, causing him or her to visit their doctor. The fatigue associated with PBC appears to be totally different from any other sort of fatigue. In early stages, many patients have commented they could sleep for hours. While some in the later stages say sleeping becomes more difficult. At this time, there is little research into the cause and treatment of the liver disease fatigue. It is usually not due to depression, and some researchers believe it is an abnormality of the axis between the pituitary and the adrenal glands. Support and understanding from family members, friends and the doctor is very important, making it somewhat bearable.
- Intense and unrelenting itching of the skin.
- Gradual darkening (hyperpigmentation) or changes in skin texture, and various skin rashes. Red color to the palms and bottom of feet.
- Small yellow or white bumps (xanthomas) under the skin, or around the eyes.
- Dry Eye Syndrome.
- Dry mouth, sometimes referred to as cotton mouth.
- Thyroid problems.
- Arthritic aches and pains in bones, muscles and joints are common. In some, the pains can be severe and debilitating. Some even report severe pain just touching leg, feet and hip bones, but this is NOT common.
Over the years, as the PBC progresses, other symptoms may appear. These symptoms may include any of the following:
- Osteoporosis or other metabolic bone disease.
- Enlarged abdomen from fluid accumulation.
- Easy bruising or bleeding.
- Jaundice (yellowing of the skin and eyes)
- Increased bilirubin.
- Internal bleeding in upper
stomach and esophagus, which may be caused by varcies.
- Hepatic encephalopathy causing personality changes: dulling of mental
functions, neglect of personal appearance, forgetfulness and trouble
concentrating, changes in sleeping habits, confusion,
breath odor and muscle stiffness. Encephalopathy
occurs in final stage of PBC.
- Hypersplenism, enlarged spleen
- Fever, nausea and vomiting.
- Reflux and stomach ulcers.
- Weight increase or decrease.
- Swelling of the hands, legs and ankles.
- Sexual problems (impotence in men, absence of periods in women, vaginal dryness and lack of desire.)
- Trembling hands.
- Difficulty in sleeping and changes in sleeping
habits. Some PBC patients have noted that itching intensifies when
they lay down to sleep. Also,
some who experience pain in the right quadrant liver area, say it is more
severe in a sleeping position.
- Hepatorenal syndrome, progressive deterioration of kidney function leading to kidney failure in a person with liver failure.
- Hepatopulmonary syndrome, associated with difficulty with breathing.
- Liver cancer (hepatocellular carcinoma)
- Abdominal pain or pressure in the liver area. Yes the
pain is real – It is believed the tissue and lining around the liver
become inflamed and the enlarged liver cause the discomforting pain.
As
the PBC progresses, some patients require vitamin A, vitamin D, vitamin E and
vitamin K replacement therapy to add back fat-soluble vitamins which are lost
in fatty stools. A calcium supplement may be prescribed to help prevent osteomalacia and osteoporosis.
When
medical treatments,
such as URSO 250, Actigall
and methotrexate, no longer control the disease, the
patient should be evaluated
for a liver transplant. The end stage
of PBC is liver failure. Many signs indicate liver failure: increased bilirubin,
jaundice,
fluid accumulation or ascites, malnutrition,
gastrointestinal bleeding, intractable itching, bone fractures and hepatic
coma. Transplant is recommended before most of these symptoms occur. The
transplant outcome for PBC patients is excellent.
As with any other chronic
illness, support and understanding are very important in helping the PBC
patient cope with the disease symptoms and day to day living.
PBC Fatigue
is the number one symptom
Fatigue is an early PBC symptom. Over time it may become severe and even debilitating.
Please help in the fight against PBC by making a tax deductible donation.
PBC Research Fund
(281) 997-1516