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Home , Common bile duct

Primary Biliary

National Digestive Diseases Information Clearinghouse

What is primary biliary Injured tissue from chronic inflamma­ tion and the buildup of leads to cirrhosis, cirrhosis? a condition in which the liver slowly deterio­ Primary biliary cirrhosis is a chronic disease rates and malfunctions. Scar tissue replaces U.S. Department that causes the bile ducts in the liver to healthy liver tissue, partially blocking the of Health and become inflamed and damaged and, ulti­ flow of blood through the liver. Scarring also Human Services mately, disappear. Bile is a liquid produced impairs the liver’s ability to in the liver that travels through the bile ducts NATIONAL • control infections INSTITUTES to the and then the small intes­ OF HEALTH tine, where it helps digest fats and fat-soluble • remove bacteria and toxins from the vitamins A, D, E, and K. When the bile blood ducts become damaged from chronic inflam­ mation, bile builds up in the liver, injuring • process nutrients, hormones, and drugs liver tissue. • make proteins that regulate blood clotting • produce bile to help absorb fats— Liver including cholesterol—and fat-soluble Bile ducts vitamins • effectively replace its own cells when they become damaged Primary biliary cirrhosis develops over time and may ultimately cause the liver to stop Cystic working completely. Most people are diag­ nosed early, before the disease progresses. Early treatment delays—but does not stop— Gallbladder the eventual onset of cirrhosis and liver failure. When a person has end-stage liver disease, a liver transplant is necessary for Common survival. Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects Normal liver and biliary system. women more often than men. What causes primary biliary What are the symptoms of cirrhosis? primary biliary cirrhosis? The cause of primary biliary cirrhosis is Most people with primary biliary cirrhosis unknown. Most research suggests the are diagnosed before symptoms begin. The disease is an autoimmune condition. The first and most common symptoms people immune system usually protects the body with this condition experience are from harmful substances such as bacteria and • a general feeling of tiredness, or fatigue viruses by attacking and destroying them. In autoimmune diseases, the immune system • pruritus—itchy skin—and darkened skin instead attacks the body’s own tissues. In in itchy areas due to scratching primary biliary cirrhosis, the immune system Other symptoms may develop, including attacks the bile ducts. • dry eyes and mouth Genetic factors may make a person prone to develop primary biliary cirrhosis. Primary • —darkening of the urine and biliary cirrhosis is more common in people yellowing of the skin and whites of the who have a parent or sibling—particularly an eyes—which occurs when the diseased identical twin—with the disease. liver does not remove enough bilirubin from the blood. Bilirubin is the pigment Genetic factors may also make some people that gives bile its reddish-yellow color. prone to develop other autoimmune dis­ eases. People with primary biliary cirrhosis may have other autoimmune conditions How is primary biliary such as rheumatoid arthritis or autoimmune cirrhosis diagnosed? thyroiditis. The first indication of primary biliary cirrho­ A person who has genetic factors for pri­ sis may occur when results of routine blood mary biliary cirrhosis may be more likely tests to check liver function are abnormal. to develop the disease after exposure to The doctor will then order one or more tests chemicals or infections, such as urinary tract to confirm the disease: infections. • Anti-mitochondrial antibody (AMA) blood test. The presence of AMA is detected in 90 percent of people with primary biliary cirrhosis. • Alkaline phosphatase blood test. Pri­ mary biliary cirrhosis is likely if two blood tests performed at least 6 months apart reveal alkaline phosphatase—a liver enzyme—to be abnormally high.

2 Primary Biliary Cirrhosis • Liver biopsy. A liver biopsy can confirm What are the complications the diagnosis but is not always neces­ sary. A biopsy may help determine of primary biliary cirrhosis? the extent of liver damage. The biopsy Some people develop one or more complica­ is performed with a needle inserted tions of primary biliary cirrhosis when the between the ribs or into a vein in the disease progresses to cirrhosis. neck. Precautions are taken to mini­ Osteoporosis. The disease may cause bones mize discomfort. A tiny sample of liver to become fragile and more likely to break. tissue is examined with a microscope Osteoporosis can also result from steroid use for scarring or other signs of cirrhosis. as a treatment of primary biliary cirrhosis. Sometimes a cause of liver damage other than cirrhosis is found during Maldigestion. When a person with primary biopsy. biliary cirrhosis has jaundice, the small intes­ tine cannot easily absorb fats and fat-soluble The doctor may also order the following vitamins. Maldigestion may result in diar­ tests: rhea and fatty stools and can lead to weight • Cholesterol blood test. People with pri­ loss in the late stages of the disease. mary biliary cirrhosis may have abnor­ . Normally, blood from mally high levels of cholesterol in the the intestines and is carried to the blood. However, these high cholesterol liver through the portal vein. But primary levels are usually less harmful to people biliary cirrhosis may cause inflammation in with primary biliary cirrhosis than to the portal tract, leading to increased pressure those without the disease. in the portal vein. This condition is called • Abdominal ultrasound. An ultrasound portal hypertension. shows whether the liver and bile ducts are inflamed. A handheld device, which and gastropathy. When a technician glides over the abdomen, portal hypertension occurs, it may cause sends sound waves toward the abdo­ enlarged blood vessels in the , men. The sound waves bounce off the called varices, or in the , called liver and other organs, and their echoes gastropathy, or both. Enlarged blood vessels create a picture of the liver and biliary are more likely to burst due to thin walls system on a video monitor. and increased pressure. If they burst, seri­ ous bleeding can occur in the esophagus or upper stomach, requiring immediate medical attention. Splenomegaly. When portal hypertension occurs, the spleen frequently enlarges and sequesters or holds white blood cells and platelets, reducing the numbers of these cells in the blood. A low platelet count in the blood may be the first evidence that a patient has developed cirrhosis.

3 Primary Biliary Cirrhosis Edema and ascites. When the liver dam- Other problems. Cirrhosis can cause age progresses to an advanced stage, fluid immune system dysfunction, leading to the collects in the legs, called edema, and in the risk of infection. Cirrhosis can also cause abdomen, called ascites. Ascites can lead to and lung failure, known as hepato- bacterial , a serious infection. renal and hepatopulmonary syndromes. Bruising and bleeding. When the liver slows or stops producing the proteins needed for How is primary biliary blood clotting, a person will bruise or bleed cirrhosis treated? easily. Early Treatment Sensitivity to medications. Cirrhosis slows Initial treatment for primary biliary cirrhosis the liver’s ability to filter medications from is aimed at relieving symptoms. Vitamin the blood. When this occurs, medications replacement therapy, calcium supplements, act longer than expected and build up in the and drugs to treat itching are usually body. This causes a person to be more sensi- prescribed. tive to medications and their side effects. A specific treatment that stops or reverses . A failing liver the progression of primary biliary cirrhosis cannot remove toxins from the blood, and has not been found. However, medication they eventually accumulate in the brain. The prescribed during the early stage of the dis- buildup of toxins in the brain—called hepatic ease may slow liver damage. encephalopathy—can decrease mental func- tion and cause coma. Signs of decreased Ursodiol (Actigall). Ursodiol is the only mental function include confusion, personal- drug approved by the U.S. Food and Drug ity changes, memory loss, trouble concentrat- Administration for the treatment of primary ing, and a change in sleep habits. biliary cirrhosis. Ursodiol assists the liver in moving bile through the ducts to the gall- Insulin resistance and type 2 diabetes. bladder and . Studies have Cirrhosis causes resistance to insulin—a shown that ursodiol prescribed early in the hormone produced by the pancreas that disease improves liver function, slowing the enables the body to use glucose as energy. time it takes to progress to and With insulin resistance, the body’s muscle, the need for a liver transplant. fat, and liver cells do not use insulin prop- erly. The pancreas tries to keep up with the Researchers are studying the effects of sev- demand for insulin by producing more, but eral other medications on the progression of excess glucose builds up in the bloodstream primary biliary cirrhosis. To date, none have causing type 2 diabetes. shown the positive effects of ursodiol. Liver cancer. is a type of liver cancer that can occur in patients with cirrhosis. Hepatocellular carcinoma has a high mortality rate, but several treatment options are available.

4 Primary Biliary Cirrhosis Treatment for Cirrhosis and Its The doctor may prescribe a beta-blocker or Complications nitrate for portal hypertension, which can lower the pressure in the varices and reduce When the disease progresses to cirrhosis, the the risk of bleeding. Gastrointestinal bleed- goals of treatment are to slow the progres- ing requires an immediate upper endoscopy sion of scar tissue in the liver and prevent to look for esophageal varices. The doctor or treat the complications of the disease. may perform a band-ligation using a special Hospitalization may be necessary for people device to compress the varices and stop the who have cirrhosis with complications. bleeding. People who have had varices in the Eating a nutritious diet. Because malnutri- past may need to take medicine to prevent tion is common in people with cirrhosis, a future episodes. healthy diet is important in all stages of the Hepatic encephalopathy is treated by cleans- disease. Health care providers recommend ing the bowel with lactulose—a laxative given a meal plan that is well balanced. If fluid orally or in enemas. Antibiotics are added to overload develops, a sodium-restricted diet the treatment if necessary. Patients may be is recommended. A person with cirrhosis asked to reduce dietary protein intake. The should not eat raw shellfish, which can con- condition may improve as other complica- tain a bacterium that causes serious infec- tions of cirrhosis are controlled. tion. To improve nutrition, the doctor may add a liquid supplement either for drinking People with cirrhosis who develop hepato- or administration with a nasogastric tube— renal syndrome must undergo regular hemo- a tiny tube inserted through the nose and dialysis treatment, which uses a machine to throat that reaches into the stomach. clean wastes from the blood. Medications are also given to improve blood flow through Avoiding alcohol and other substances. the kidneys. People with primary biliary cirrhosis are encouraged not to consume alcohol or Liver Transplantation illicit substances, as both will cause more A liver transplant is the only treatment that liver damage. Because many vitamins and will cure primary biliary cirrhosis. A liver medications—prescription and over-the- transplant is considered when complications counter—can affect liver function, a doctor cannot be controlled by treatment. A liver should be consulted before taking them. transplant is a major operation in which the Treatment for cirrhosis also addresses spe- diseased liver is removed and replaced with a cific complications. For edema and ascites, healthy one from an organ donor. A team of the doctor may recommend diuretics— health professionals determines the risks and medications that remove fluid from the benefits of the procedure for each patient. body. Large amounts of ascitic fluid may be Survival rates have improved over the past removed from the abdomen and checked for several years because of drugs that suppress bacterial peritonitis. Oral antibiotics may the immune system and keep it from attack- be prescribed to prevent infection. Severe ing and damaging the new liver. infection with ascites requires intravenous (IV) antibiotics.

5 Primary Biliary Cirrhosis The number of people who need a liver • Many people with primary biliary cir­ transplant far exceeds the number of avail­ rhosis do not have symptoms until after able organs. A person needing a transplant the disease is diagnosed. must go through a complicated evaluation • The first and most common symp­ process before being added to a long trans­ toms of the disease are itching, called plant waiting list. Generally, organs are pruritus, and fatigue. Other symp­ given to people with the best chance of living toms include dry eyes and mouth and the longest after a transplant. Survival after jaundice. a transplant requires intensive follow-up and cooperation on the part of the patient and • The anti-mitochondrial antibody caregiver. (AMA) blood test, the alkaline phos­ phatase blood test, and a liver biopsy may be necessary to confirm a diagnosis Points to Remember of primary biliary cirrhosis. • Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the • Some of the complications of primary liver to become inflamed and damaged biliary cirrhosis are osteoporosis and and, ultimately, disappear. maldigestion. • Injured liver tissue from chronic inflam­ • Early treatment delays—but does not mation and the buildup of bile leads to stop—the eventual onset of cirrhosis cirrhosis, a condition in which the liver and liver failure. slowly deteriorates and malfunctions. • Ursodiol (Actigall) is the only drug • The cause of primary biliary cirrhosis is approved by the U.S. Food and Drug unknown. Most research suggests the Administration for the treatment of disease is an autoimmune condition. primary biliary cirrhosis. • Primary biliary cirrhosis is more com­ • Liver transplantation is the only treat­ mon in people who have a parent or ment that will cure primary biliary sibling—particularly an identical twin— cirrhosis. A liver transplant is consid­ with the disease. ered when complications cannot be controlled by treatment. • Most people are diagnosed early, before the disease progresses. The disease is often discovered when routine blood tests to check liver function are abnormal.

6 Primary Biliary Cirrhosis Hope through Research For More Information The National Institute of Diabetes and American Liver Foundation Digestive and Kidney Diseases’ Division of 75 Maiden Lane, Suite 603 Digestive Diseases and Nutrition supports New York, NY 10038 basic and clinical research into liver Phone: 1–800–GO–LIVER (465–4837), diseases—including primary biliary 1–888–4HEPUSA (443–7872), cirrhosis—and liver transplantation. or 212–668–1000 Researchers are studying Fax: 212–483–8179 Email: [email protected] • the mechanisms of liver injury and Internet: www.liverfoundation.org regeneration United Network for Organ Sharing • ways to improve outcomes of liver P.O. Box 2484 transplantation Richmond, VA 23218 • how autoimmune liver diseases develop Phone: 1–888–894–6361 in the body or 804–782–4800 Participants in clinical trials can play a more Fax: 804–782–4817 active role in their own health care, gain Internet: www.unos.org access to new research treatments before they are widely available, and help others by contributing to medical research. For infor­ mation about current studies, visit www.ClinicalTrials.gov.

7 Primary Biliary Cirrhosis You may also find additional information about this National Digestive Diseases topic by visiting MedlinePlus at www.medlineplus.gov. Information Clearinghouse This publication may contain information about med- ications. When prepared, this publication included 2 Information Way the most current information available. For updates Bethesda, MD 20892–3570 or for questions about any medications, contact the U.S. Food and Drug Administration toll-free at Phone: 1–800–891–5389 1–888–INFO–FDA (463–6332) or visit www.fda.gov. TTY: 1–866–569–1162 Consult your doctor for more information. Fax: 703–738–4929 Email: [email protected] Internet: www.digestive.niddk.nih.gov

The U.S. Government does not endorse or favor any The National Digestive Diseases Information specific commercial product or company. Trade, Clearinghouse (NDDIC) is a service of the proprietary, or company names appearing in this National Institute of Diabetes and Digestive document are used only because they are considered necessary in the context of the information provided. and Kidney Diseases (NIDDK). The NIDDK If a product is not mentioned, the omission does not is part of the National Institutes of Health of mean or imply that the product is unsatisfactory. the U.S. Department of Health and Human Services. Established in 1980, the Clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. The NDDIC answers inquiries, develops and distributes publications, and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases. Publications produced by the Clearinghouse are carefully reviewed by both NIDDK scientists and outside experts. This publication was reviewed by M. Eric Gershwin, M.D., University of California at Davis.

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U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health

NIH Publication No. 09–4625 December 2008