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Primary Biliary

National Digestive Diseases Information Clearinghouse

What is primary biliary cirrhosis? Primary biliary cirrhosis is a chronic, or long lasting, disease that causes the small ducts in the to become inflamed and damaged and ultimately disappear. The bile ducts carry a fluid called bile from the liver to the , where it is stored. When food enters the stomach after a meal, the gallbladder contracts, and the bile ducts carry bile to the , the first part of the , for use in digestion. The liver makes bile, which is made up of Liver bile acids, , , and fluids. Bile helps the body absorb fats, cholesterol, and -soluble vitamins. Bile also carries Bile ducts cholesterol, toxins, and waste products to the intestines, where the body removes them. Gallbladder When chronic , or swelling, damages the bile ducts, bile and toxic wastes Duodenum build up in the liver, damaging liver tissue.

This damage to the liver tissue can lead Primary biliary cirrhosis causes the small bile ducts to cirrhosis, a condition in which the liver in the liver to become inflamed and damaged and slowly deteriorates and is unable to function ultimately disappear. normally. In cirrhosis, scar tissue replaces healthy liver tissue, partially blocking the flow of blood through the liver. The liver is the body’s largest internal organ. What causes primary biliary The liver is called the body’s metabolic factory because of the important role it cirrhosis? plays in —the way cells change The causes of primary biliary cirrhosis are food into energy after food is digested and unknown. Most research suggests it is an absorbed into the blood. The liver has many autoimmune disease. The immune system functions, including protects people from by identifying and destroying bacteria, viruses, and other • taking up, storing, and processing potentially harmful foreign substances. An nutrients from food—including fat, autoimmune disease is a disorder in which sugar, and —and delivering them the body’s immune system attacks the body’s to the rest of the body when needed own cells and organs. In primary biliary • making new , such as clotting cirrhosis, the immune system attacks the factors and immune factors small bile ducts in the liver. • producing bile Genetics, or inherited genes, can make a person more likely to develop primary biliary • removing waste products the kidneys cirrhosis. Primary biliary cirrhosis is more cannot remove, such as fats, cholesterol, common in people who have a parent or toxins, and medications sibling—particularly an identical twin—with A healthy liver is necessary for survival. The the disease. In people who are genetically liver can regenerate most of its own cells more likely to develop primary biliary when they become damaged. However, cirrhosis, environmental factors may trigger if injury to the liver is too severe or long or worsen the disease, including lasting, regeneration is incomplete, and the liver creates scar tissue. Scarring of the liver • exposure to toxic chemicals may lead to cirrhosis. • smoking The buildup of scar tissue that causes • cirrhosis is usually a slow and gradual Genetics can also make some people more process. In the early stages of cirrhosis, the likely to develop other autoimmune diseases, liver continues to function. However, as such as cirrhosis gets worse and scar tissue replaces more healthy tissue, the liver will begin to • autoimmune , a disease in fail. Chronic , which is also called which the body’s immune system attacks end-stage , progresses over liver cells months, years, or even decades. With end- • Sjögren’s syndrome, a condition in stage liver disease, the liver can no longer which the immune system attacks the perform important functions or effectively glands that produce tears and saliva replace damaged cells. • autoimmune thyroid dysfunctions, Primary biliary cirrhosis usually occurs conditions in which the immune system between the ages of 30 and 65 and affects attacks the thyroid gland women more often than men.1

1Pyrsopoulos NT. Primary biliary cirrhosis. Medscape website. http://emedicine.medscape.com/ article/171117-overview. Updated May 20, 2013. Accessed February 7, 2014. 2 Primary Biliary Cirrhosis What are the symptoms is a common of cirrhosis. This condition may lead to other of primary biliary cirrhosis? complications, such as The first and most common symptoms of primary biliary cirrhosis are • —swelling due to a buildup of fluid—in the feet, ankles, or legs, • , or feeling tired and —a buildup of fluid in the • itching skin, and darkened skin in itching areas due to scratching • enlarged blood vessels, called varices, in • dry eyes and mouth the , stomach, or both Some people may have , a condition • an enlarged spleen, called that causes the skin and whites of the eyes to • mental confusion due to a buildup of turn yellow. Health care providers diagnose toxins that are ordinarily removed by up to 60 percent of people with primary the liver, a condition called hepatic biliary cirrhosis before symptoms begin.2 Routine blood tests showing abnormal liver enzyme levels may lead a health care Edema and ascites. Liver failure causes provider to suspect that a person without fluid buildup that results in edema and symptoms has primary biliary cirrhosis. ascites. Ascites can lead to spontaneous bacterial , a serious infection that requires immediate medical attention. What are the complications Varices. may cause of primary biliary cirrhosis? enlarged blood vessels in the esophagus, Most complications of primary biliary stomach, or both. These enlarged blood cirrhosis are related to cirrhosis and start vessels, called esophageal or , after primary biliary cirrhosis progresses to cause the vessel walls to become thin and cirrhosis. In some cases, portal hypertension to increase, making the and may develop before blood vessels more likely to burst. If they cirrhosis. burst, serious bleeding can occur in the Portal hypertension. The portal carries esophagus or upper stomach, requiring blood from the stomach, intestines, spleen, immediate medical attention. gallbladder, and pancreas to the liver. In Splenomegaly. Portal hypertension may cirrhosis, scar tissue partially blocks the cause the spleen to enlarge and retain normal flow of blood, which increases the white blood cells and platelets, reducing pressure in the portal vein. This condition the numbers of these cells and platelets in is called portal hypertension. Portal the blood. A low platelet count may be the first evidence that a person has developed cirrhosis.

2Angulo P, Lindor KD. Chapter 89: primary biliary cirrhosis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran’s Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 9th ed. Philadelphia: Saunders Elsevier; 2010: 1477–1488. 3 Primary Biliary Cirrhosis . A failing liver . Steatorrhea is a condition in cannot remove toxins from the blood, so which the body cannot absorb fat, causing they eventually accumulate in the brain. a buildup of fat in the stool and loose, The buildup of toxins in the brain is called greasy, and foul-smelling bowel movements. hepatic encephalopathy. This condition Steatorrhea may be caused by impairment can decrease mental function and cause of bile delivery to the small intestine or by and even . Stupor is an the pancreas not producing enough digestive unconscious, sleeplike state from which enzymes. a person can only be aroused briefly by Liver cancer. Liver cancer is common in a strong stimulus, such as a sharp pain. people with cirrhosis. Liver cancer has a Coma is an unconscious, sleeplike state high mortality rate. Current treatments are from which a person cannot be aroused. limited and only fully successful if a health Signs of decreased mental function include care provider detects the cancer early, before • confusion the tumor is too large. For this reason, health care providers should check people • personality changes with cirrhosis for signs of liver cancer every • memory loss 6 to 12 months. Health care providers use • trouble concentrating blood tests, , or both to check for signs of liver cancer. • a change in sleep habits Metabolic bone diseases. Some people with How is primary biliary cirrhosis develop a metabolic bone disease, which is a disorder of bone strength usually cirrhosis diagnosed? caused by abnormalities of vitamin D, bone A health care provider may use the following mass, bone structure, or minerals, such as tests to diagnose primary biliary cirrhosis: and phosphorous. Osteopenia is a • a medical and family history condition in which the bones become less dense, making them weaker. When bone • a physical exam loss becomes more severe, the condition • blood tests is referred to as osteoporosis. People with these conditions are more likely to develop • imaging tests bone fractures. • a and stones. If cirrhosis A health care provider usually bases a prevents bile from flowing freely to and diagnosis of primary biliary cirrhosis on two from the gallbladder, the bile hardens into out of three of the following criteria: gallstones. Symptoms of gallstones include • a showing elevated liver and recurrent bacterial enzymes cholangitis—irritated or infected bile ducts. Stones may also form in and block the bile • a blood test showing the presence of ducts, causing pain, jaundice, and bacterial anti-mitochondrial antibodies (AMA) cholangitis. • a liver biopsy showing signs of the disease

4 Primary Biliary Cirrhosis Health care providers may order additional Imaging tests. A health care provider may tests to rule out other causes of symptoms. use the following imaging tests to examine Health care providers diagnose the majority the bile ducts. These tests can distinguish of people with primary biliary cirrhosis early between primary biliary cirrhosis and other in the course of the disease. conditions that affect the bile ducts. Medical and family history. Taking a • Ultrasound uses a device, called a medical and family history is one of the first transducer, that bounces safe, painless things a health care provider may do to help sound waves off organs to create an diagnose primary biliary cirrhosis. He or she image of their structure. A specially will ask a patient to provide a medical and trained technician performs the family history. procedure in a health care provider’s office, an outpatient center, or a Physical exam. A physical exam may help hospital, and a radiologist—a doctor diagnose primary biliary cirrhosis. During a who specializes in medical imaging— physical exam, a health care provider usually interprets the images. A patient does • examines a patient’s body not need anesthesia. In addition to showing problems with the bile ducts, • uses a stethoscope to listen to sounds in the images can show signs of advanced the abdomen cirrhosis or complications. • taps on specific areas of the patient’s • Magnetic resonance body cholangiopancreatography uses The health care provider will perform a magnetic resonance imaging (MRI) physical exam to look for signs of the disease. to examine the bile ducts. MRI For example, the liver may feel hard or machines use radio waves and magnets ascites may cause the abdomen to enlarge. to produce detailed pictures of the body’s internal organs and soft tissues Blood test. A blood test involves drawing without using x rays. A specially blood at a health care provider’s office or a trained technician performs magnetic commercial facility and sending the sample resonance cholangiopancreatography to a lab for analysis. The blood test can in an outpatient center or a hospital, show elevated levels of liver enzymes, such and a radiologist interprets the images. as alkaline phosphatase. A routine blood A patient does not need anesthesia, test may show high levels of the liver enzyme though a health care provider may use alkaline phosphatase in people who have light sedation for patients with a fear primary biliary cirrhosis and are not yet of confined spaces. With most MRI showing symptoms. machines, the patient lies on a table that The health care provider will perform slides into a tunnel-shaped device that an AMA blood test to help confirm the may be open ended or closed at one diagnosis. A blood test will detect the end; some machines allow the patient to presence of AMA in 90 to 95 percent of lie in a more open space. people with primary biliary cirrhosis.3

3Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. AASLD practice guidelines: primary biliary cirrhosis. . 2009;50(1):291–308. 5 Primary Biliary Cirrhosis • Endoscopic retrograde During the procedure, the patient lies on cholangiopancreatography uses an a table, right hand resting above the head. x ray to look at the bile ducts. A The health care provider applies a local gastroenterologist—a doctor who anesthetic to the area where he or she will specializes in digestive diseases— insert the biopsy needle. If needed, a health performs the test at a hospital or an care provider will also give and outpatient center. After lightly sedating pain medication. The health care provider the patient, the gastroenterologist uses a needle to take a small piece of liver inserts an endoscope—a small, tissue. He or she may use ultrasound, flexible tube with a light and a camera computerized tomography scans, or other on the end—through the mouth imaging techniques to guide the needle. into the duodenum and bile ducts. After the biopsy, the patient must lie on the The endoscope is connected to a right side for up to 2 hours and is monitored computer and video monitor. The an additional 2 to 4 hours before being sent gastroenterologist injects a special dye, home. called contrast medium, through the tube into the bile ducts, which makes A health care provider performs a liver the ducts show up on the monitor. This biopsy at a hospital or an outpatient test is more invasive than other imaging center. The health care provider sends tests, and health care providers do not the liver sample to a pathology lab, where routinely need the test to make the the pathologist—a doctor who specializes diagnosis of primary biliary cirrhosis. in diagnosing diseases—looks at the tissue A health care provider uses the test with a microscope and sends a report to the selectively when he or she is concerned patient’s health care provider. that the blockage of the bile ducts has A liver biopsy can confirm the diagnosis of another cause, such as a or a primary biliary cirrhosis; however, a person narrowing of the large bile ducts due to does not always need this test. A health inflammation or cancer. Patients may care provider will perform a biopsy if the have pain, , or vomiting after the AMA blood test is negative and the person test or may develop bacterial cholangitis shows other signs of primary biliary cirrhosis. or —inflammation of the Sometimes a health care provider finds a pancreas. cause of liver damage other than primary Liver biopsy. A liver biopsy is a procedure biliary cirrhosis during biopsy. that involves taking a piece of liver tissue for examination with a microscope for signs of damage or disease. The health care provider may ask the patient to stop taking certain medications temporarily before the liver biopsy. The health care provider may ask the patient to fast for 8 hours before the procedure.

6 Primary Biliary Cirrhosis Liver

A liver biopsy is a procedure that involves taking a piece of liver tissue for examination with a microscope for signs of damage or disease.

How is primary biliary Medications cirrhosis treated? Health care providers prescribe ursodiol Treatment for primary biliary cirrhosis (Actigall, Urso) to treat primary biliary depends on how early a health care cirrhosis. Ursodiol is a nontoxic provider diagnoses the disease and whether that people can take orally. Ursodiol complications are present. In the early replaces the bile acids that are normally stages of primary biliary cirrhosis, treatment produced by the liver, which are more toxic can slow the progression of liver damage to and can harm the liver. Treatment with cirrhosis. In the early stages of cirrhosis, ursodiol can reduce levels of and the goals of treatment are to slow the liver enzymes in the blood. Early treatment progression of tissue scarring in the liver with this medication reduces the likelihood and prevent complications. As cirrhosis of needing a liver transplant and improves 3 progresses, a person may need additional survival. Early treatment provides the most treatments and hospitalization to manage benefit; however, ursodiol treatment late in complications. the course of the disease can still slow the progression of liver damage. While ursodiol treatment improves the outcome of primary biliary cirrhosis, it does not cure the disease.

7 Primary Biliary Cirrhosis Researchers are studying the effects of or cevimeline (Evoxac). People who several other medications on the progression have difficulty with dry eyes should of primary biliary cirrhosis. To date, none see an ophthalmologist—a doctor who has shown the positive effects of ursodiol. diagnoses and treats all eye diseases and eye disorders—regularly. People with dry Avoiding Alcohol and Other mouth should have regular dental exams. Substances Portal hypertension. A health care provider People with cirrhosis should not drink any may prescribe a beta-blocker or nitrate to alcohol or take any illegal substances, as treat portal hypertension. Beta-blockers both will cause more liver damage. People lower blood pressure by helping the heart with cirrhosis should avoid complementary beat slower and with less force, and nitrates and alternative medications, such as herbs. relax and widen blood vessels to let more People with cirrhosis should be careful blood flow to the heart and reduce the about starting new medications and should heart’s workload. consult a health care provider before taking prescription medications, over-the-counter Varices. Beta-blockers can lower the medications, or vitamins. Many vitamins pressure in varices and reduce the likelihood and prescription and over-the-counter of bleeding. Bleeding in the stomach or medications can affect liver function. esophagus requires an immediate upper endoscopy. This procedure involves using Treatment of Symptoms an endoscope to look for varices. The and Complications health care provider may use the endoscope to perform a band ligation, a procedure Health care providers treat symptoms and that involves placing a special rubber band complications as follows: around the varices that causes the tissue Itching. Antihistamines may help with mild to die and fall off. A gastroenterologist itching. However, antihistamines often performs the procedure at a hospital or cause drowsiness, and a person should an outpatient center. People who have take antihistamines just before bedtime to had varices in the past may need to take help with nighttime itching. A health care medication to prevent future episodes. provider will treat more problematic itching Edema and ascites. Health care providers with cholestyramine (Locholest, Questran), prescribe —medications that which reduces cholesterol in the blood. remove fluid from the body—to treat edema Experts believe high levels of cholesterol and ascites. A health care provider may let substances that cause itching build up in remove large amounts of ascitic fluid from tissues. the abdomen and check for spontaneous Dry eyes and mouth. Health care providers bacterial peritonitis. A health care provider usually treat dry eyes and mouth with may prescribe bacteria-fighting medications artificial tears and saliva substitutes, called to prevent infection. He or respectively. These products are available she may prescribe oral antibiotics; however, without a prescription. A health care severe infection with ascites requires provider may treat people whose symptoms intravenous (IV) antibiotics. do not improve with pilocarpine (Salagen)

8 Primary Biliary Cirrhosis Hepatic encephalopathy. A health care replace it with a healthy liver or part of a provider will treat hepatic encephalopathy liver from another person, called a donor. by cleansing the bowel with , a Read more in at given orally or as an —a www.digestive.niddk.nih.gov. liquid put into the . A health care provider may also add antibiotics to the treatment. Hepatic encephalopathy may Eating, Diet, and Nutrition improve as other complications of cirrhosis A is important in all stages of are controlled. cirrhosis because malnutrition is common in Osteoporosis. A health care provider may people with this disease. Malnutrition is a prescribe bisphosphonate medications to condition that occurs when the body does not improve bone density. get enough nutrients. Cirrhosis may lead to malnutrition because it can cause Gallstones and bile duct stones. A health care provider may use to remove • people to eat less because of symptoms gallstones. He or she may use endoscopic such as loss of appetite retrograde cholangiopancreatography, which • changes in metabolism uses balloons and basketlike devices, to • reduced absorption of vitamins and retrieve the bile duct stones. minerals Liver cancer. A health care provider Health care providers can recommend a may recommend screening tests every meal plan that is well balanced and provides 6 to 12 months to check for signs of liver enough calories and protein. If ascites cancer. Screening tests can find cancer develops, a health care provider or dietitian before the person has symptoms of the may recommend a -restricted diet. To disease. Cancer treatment is usually more improve nutrition, the health care provider effective when the health care provider finds may prescribe a liquid supplement. A person the disease early. Health care providers use may take the liquid by mouth or through blood tests, ultrasound, or both to screen for a nasogastric tube—a tiny tube inserted liver cancer in people with cirrhosis. He or through the nose and throat that reaches into she may treat cancer with a combination of the stomach. surgery, radiation, and chemotherapy. A person with cirrhosis should not eat raw When is a liver transplant shellfish, which can contain a bacterium that causes serious infection. Cirrhosis affects the considered for primary immune system, making people with cirrhosis biliary cirrhosis? more likely than healthy people to develop A health care provider may consider a liver an infection after eating shellfish that contain transplant when cirrhosis leads to liver this bacterium. failure or treatment for complications is A health care provider may recommend ineffective. Liver transplantation is surgery calcium and vitamin D supplements to help to remove a diseased or an injured liver and prevent osteoporosis.

9 Primary Biliary Cirrhosis Points to Remember • Health care providers prescribe ursodiol (Actigall, Urso) to treat primary biliary • Primary biliary cirrhosis is a chronic cirrhosis. Early treatment with this disease that causes the small bile ducts medication reduces the likelihood of in the liver to become inflamed and needing a liver transplant and improves damaged and ultimately disappear. survival. • When chronic inflammation damages • A health care provider may consider the bile ducts, bile and toxic wastes a liver transplant when cirrhosis build up in the liver, damaging liver leads to liver failure or treatment for tissue. This damage to the liver tissue complications is ineffective. can lead to cirrhosis. • The causes of primary biliary cirrhosis Hope through Research are unknown. Most research suggests it is an autoimmune disease. The National Institute of and Digestive and Kidney Diseases’ (NIDDK’s) • Primary biliary cirrhosis is more Division of Digestive Diseases and Nutrition common in people who have a parent or supports basic and clinical research into sibling—particularly an identical twin— liver diseases—including primary biliary with the disease. cirrhosis—and liver transplantation. • The first and most common symptoms Clinical trials are research studies involving of primary biliary cirrhosis are fatigue, people. Clinical trials look at safe and itching, and dry eyes and mouth. Some effective new ways to prevent, detect, or people may have jaundice, a condition treat disease. Researchers also use clinical that causes the skin and whites of trials to look at other aspects of care, such the eyes to turn yellow. Health care as improving the quality of life for people providers diagnose up to 60 percent of with chronic illnesses. To learn more about people with primary biliary cirrhosis clinical trials, why they matter, and how to before symptoms begin. participate, visit the NIH Clinical Research • Most complications of primary biliary Trials and You website at www.nih.gov/health/ cirrhosis are related to cirrhosis and clinicaltrials. For information about current start after primary biliary cirrhosis studies, visit www.ClinicalTrials.gov. progresses to cirrhosis. • A health care provider may use the following tests to diagnose primary biliary cirrhosis: − a medical and family history − a physical exam − blood tests − imaging tests − a liver biopsy

10 Primary Biliary Cirrhosis For More Information You may also find additional information about this American Liver Foundation topic by visiting MedlinePlus at www..gov. This publication may contain information about 39 Broadway, Suite 2700 medications and, when taken as prescribed, New York, NY 10006 the conditions they treat. When prepared, this Phone: 1–800–GO–LIVER publication included the most current information available. For updates or for questions about (1–800–465–4837) or 212–668–1000 any medications, contact the U.S. Food and Drug Fax: 212–483–8179 Administration toll-free at 1–888–INFO–FDA Internet: www.liverfoundation.org (1–888–463–6332) or visit www.fda.gov. Consult your health care provider for more information. United Network for Organ Sharing P.O. Box 2484 The U.S. Government does not endorse or favor any Richmond, VA 23218 specific commercial product or company. Trade, Phone: 1–888–894–6361 or 804–782–4800 proprietary, or company names appearing in this Fax: 804–782–4817 document are used only because they are considered necessary in the context of the information provided. Internet: www.unos.org If a product is not mentioned, the omission does not mean or imply that the product is unsatisfactory. Acknowledgments Publications produced by the Clearinghouse are carefully reviewed by both NIDDK scientists and outside experts. This publication was originally reviewed by M. Eric Gershwin, M.D., University of California at Davis. Gregory Gores, M.D., and Jayant A. Talwalkar, M.D., M.P.H., both from the Mayo Clinic, reviewed the updated version of the publication.

11 Primary Biliary Cirrhosis National Digestive Diseases Information Clearinghouse 2 Information Way Bethesda, MD 20892–3570 Phone: 1–800–891–5389 TTY: 1–866–569–1162 Fax: 703–738–4929 Email: [email protected] Internet: www.digestive.niddk.nih.gov The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health of 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.

This publication is not copyrighted. The Clearinghouse encourages users of this publication to duplicate and distribute as many copies as desired. This publication is available at www.digestive.niddk.nih.gov.

NIH Publication No. 14–4625 March 2014

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