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Primary Biliary Cirrhosis

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Primary Biliary Cirrhosis
Primary biliary cirrhosis (PBC) is a disease of the small bile ducts in your liver. It is a chronic disease which means it is ongoing. It leads to liver inflammation and eventually, to a disease called cirrhosis.
For most people, PBC progresses slowly and they can live active, productive lives.
Read this information to
understand:
• How your liver and bile ducts work.
• How PBC is diagnosed.
• How to manage the symptoms of PBC.
• The importance of follow-up care.

How the Liver Works
The liver is the largest solid organ in your body. Fluid produced by the liver is called bile. Bile travels through the liver ducts and empties into your intestines to help with the digestion of fat in your diet (Figure 1). Bile also carries waste products, such as cholesterol and bilirubin, out of the liver.
About Primary Biliary Cirrhosis
In the early stages of PBC, the small bile ducts and the tissues around them become inflamed. As the inflammation gets worse, scar tissue forms which then blocks the bile ducts. As scar tissue builds up in the bile ducts, it becomes more difficult for the liver to work like it should.
The inflammation and scar tissue buildup leads to cirrhosis, a condition in which scar tissue affects blood flow through the liver and keeps it from working normally.
Who gets PBC?
PBC is most often diagnosed in women in their 40s and 50s. Women have it 10 times more often than men. It is slightly more common among siblings and in families where one member has had the disease. The disease is uncommon, but the number of people diagnosed with it is rising.
Symptoms of PBC
Many people with PBC have no symptoms at all. The disease may be diagnosed during a routine health exam or when a blood test is done for another reason. After diagnosis, many people have no symptoms for years.

Early symptoms may include:
• Feeling tired.
• Itching on the palms of your hands and soles of your feet. Some have itching everywhere.
• Dry eyes and mouth.
As the disease progresses, symptoms may include:
• Yellowing of the skin and eyes, known as jaundice.
• Gastrointestinal bleeding.
• A blotchy, slightly tanned appearance to the skin, called hyperpigmentation.
• Excess fluid in the abdomen, called ascites.
• Swelling, called edema, particularly in your ankles.
• Waxy bumps that most often appear on the eye’s upper and lower lids.
Causes of PBC
The exact cause of PBC is unknown. The most likely cause is a problem with your immune system fighting against the cells lining the bile ducts. This immune system response may be due to something in the environment, a virus or bacteria, or something in your genetic makeup.
Research is ongoing about the cause of PBC.
How PBC Is Diagnosed
Diagnosis is usually based on a combination of tests which may include these:
Blood tests
Blood tests may be done to look for:
Alkaline phosphatase — An enzyme made in the bile ducts. If your level is higher than normal, this can mean you have inflammation in the bile ducts.
Antimitochondrial antibody (AMA) — Almost all people who have PBC have this antibody in their blood.
Liver biopsy
If your provider suspects you have PBC, but the blood test for AMA is negative, he or she may request you have a liver biopsy. During a liver biopsy, a small piece of tissue is taken from your liver using a special needle. Imaging exams such as ultrasound or CT scans are often used to guide the placement of the needle.
A biopsy can show what stage of the disease you are in. You may have read or heard about the stages of PBC, but unlike staging for other diseases, staging is not an important factor in the diagnosis or treatment of PBC.
PBC and Other Diseases
If you have PBC, you are more likely to have the following conditions as well:
Sicca syndrome
This is a condition in which the tear glands along your eyelids and the salivary glands in your mouth do not produce enough moisture. This is why you may have dry eyes and mouth. Artificial tears may be helpful to prevent dry eyes. For dry mouth, try using a saliva substitute such as Moi-stir™ or Salivent™.
Thyroid disorders
The thyroid gland is located at the base of your neck. It helps set the rate at which your body functions. If you have PBC, your thyroid gland may slow your body’s normal functioning, called hypothyroidism. Your health care provider can prescribe medicines to help treat this condition.
Osteoporosis
PBC can cause you to lose bone density twice as fast as normal. Bones can become thin and brittle and are more likely to break. To prevent bone loss, your health care provider can give you suggestions about taking enough calcium, doing weight-bearing activities and exercising, or taking other medicines.
Inflammatory arthritis
About 15 percent of people with PBC have joint aches caused by joint inflammation.
Complications of PBC
High cholesterol
Cholesterol is normally excreted in the bile. If your bile ducts are blocked, the cholesterol cannot be excreted.
Portal hypertension
High blood pressure in the blood vessels that line the esophagus and stomach is called portal hypertension. Blood from your intestine, spleen and pancreas enters through your liver through a large blood vessel called the portal vein. When scar tissue blocks normal circulation through your liver, blood backs up, leading to increased pressure within the vein. These vessels may rupture and bleed. This tends to happen if you have advanced PBC.
Primary liver cancer
Liver cancer is cancer that begins in the cells of your liver. PBC causes scar tissue to form in your liver which increases your chances of developing liver cancer.
Treatment for PBC
There is no cure for PBC, however, medicines are available to slow the disease’s progression and to manage the symptoms. Your health care team’s goal is to manage the symptoms of the disease while trying to improve or maintain your daily functioning and quality of life.
Medicine to slow the disease’s progression
Ursodeoxycholic acid — This medicine can help reduce the liver enzymes that are usually higher such as alkaline phosphatase.
Medicines to manage symptoms or prevent complications
Medicines to manage itching include cholestyramine, diphenhydramine, and rifampin.
Non-selective beta blockers are used if you have high blood pressure in the blood vessels around the liver.
Vitamin supplements are used to replace the vitamins your body is missing.
Liver transplant
Your health care team closely watches your symptoms and test results to see if your PBC is getting worse. Your team may recommend that you have a liver transplant if your disease progresses. Reasons your team may recommend a liver transplant include:
• You have a buildup of fluid in the abdomen.
• You have changes in your ability to think clearly.
• You have bleeding in the intestines.
• You have jaundice.
• You have uncontrolled itching.
• You have liver cancer.
• Your kidney is not functioning well
Caring for Yourself When You Have PBC
If you have PBC, there are steps you can take do care for yourself that can improve your overall health such as:
• Avoid drinking alcohol. If you have questions, talk with your health care team.
• Get vaccinated against hepatitis A and B.
• If you smoke, quit. If you don’t smoke, don’t start.
• Choose a healthy diet full of fruits and vegetables and whole grains. Ask your health care team about meeting with a dietitian if you need help setting up a healthy diet.
• Maintain a healthy weight.
• Talk to your health care team about taking over-the-counter medicines. You may be asked to limit or not take certain medicines.
• Exercise such as walking 30 minutes most days of the week.
• Spend time with family and friends.
• Use stress management techniques such as yoga, meditation and relaxation exercises.
Follow-Up Care
When you have PBC, follow-up care is critical. Be sure to see your health care providers every 12 months. This can help your MIDAS health care team monitor your condition and help you manage the disease.
You may need to have appointments more often if you are having symptoms of complications or if the disease is advancing or your health care team recommends it.
You need to schedule your follow-up appointments; they are not automatically made for you. However, if you have tests done at MIDAS, you may be contacted a few days later about scheduling an appointment to talk about the results.
Living With PBC
How PBC progresses varies from person to person. Some people do well for many years with no problems, while others have complications such as itching or edema. These and other complications can usually be managed.
For most people, the disease does not affect their day-to-day activities. People with PBC work, go to school and generally enjoy a good quality of life.
Complications related to PBC may make it hard for you to continue normal activities and some may shorten how long you live. Most people with PBC, however, enjoy good health for many years.
The most important thing you can do if you have PBC is have a partnership with a health care provider who has expertise in treating PBC. Attend all health care visits, know the symptoms of complications and disease progression, and tell your provider about any changes or concerns.
Medical Research
While the cause for PBC is unknown, medical research has helped health care providers understand more about the disease. In the future, clinical trials may be available to test new therapies. Ask your health care provider if you would like more information about PBC research.
Resources
For more information, go to Midas clinic.com or ask your health care provider to suggest websites. The following websites, which Midas Clinic does not sponsor or endorse, are other options. MIDAS cannot guarantee the accuracy of the information on these sites. Websites should not be used to diagnose or treat health conditions.
Contacting Your Health Care Team
The symptoms listed here may mean you are having complications of PBC or that your PBC is progressing. Know these symptoms so you can act quickly.
Contact your health care provider within 48 hours if you have:
• Jaundice.
• Abdominal pain.
• Itching that is getting worse.
Call or seek emergency medical care if:
• You are throwing up blood or you have bloody, black or dark stools.
• You have unexplained confusion.
• Your abdomen or feet are gradually becoming more swollen.
Conclusion
Finding out that you have an ongoing disease like PBC can be upsetting. Know that you do not have to manage alone. Your health care team is here to help. Understanding the terms and knowing what to expect can take some of the stress away. Just by reading this brochure, you have taken an important step in preparing yourself for dealing with PBC.
If you have questions about PBC or the information in this brochure, talk with a member of your health care team at Midas.