BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuPrimary biliary cirrhosisPrimary biliary cholangitis; PBCThe bile ducts are tubes that move bile from the liver to the small intestine. Bile is a substance that helps with digestion. All of the bile ducts together are called the biliary tract.BileBile is a fluid that is made and released by the liver and stored in the gallbladder. Bile helps with digestion. It breaks down fats into fatty acid...ImageRead Article Now Book Mark Article When the bile ducts become swollen or inflamed, this blocks the flow of bile. These changes can lead to scarring of the liver called cirrhosis. This is called biliary cirrhosis. Advanced cirrhosis can lead to liver failure. Causes The cause of inflamed bile ducts in the liver is not known. However, primary biliary cirrhosis is an autoimmune disorder. That means your body's immune system mistakenly attacks healthy tissue. The disease may be linked to autoimmune disorders such as:Autoimmune disordersAn autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 types of aut...ImageRead Article Now Book Mark Article Celiac disease Celiac diseaseCeliac disease is an autoimmune condition that damages the lining of the small intestine. This damage comes from a reaction to eating gluten. This ...ImageRead Article Now Book Mark Article Raynaud phenomenon Raynaud phenomenonRaynaud phenomenon is a condition in which cold temperatures or strong emotions cause blood vessel spasms. This blocks blood flow to the fingers, to...ImageRead Article Now Book Mark Article Sicca syndrome (dry eyes or mouth) Thyroid disease The disease most often affects middle-aged women. Symptoms More than one half of people have no symptoms at the time of diagnosis. Symptoms most often begin slowly. Early symptoms may include: Nausea and belly pain Fatigue and loss of energy Fatty deposits under the skin Fatty stools Itching Poor appetite and weight lossAs liver function worsens, symptoms may include:Fluid buildup in the legs (edema) and in the abdomen (ascites) Yellow color in the skin, mucous membranes, or eyes (jaundice) Redness on the palms of the hands In men, impotence, shrinking of the testicles, and breast swelling Easy bruising and abnormal bleeding, most often from swollen veins in the digestive tract Confusion or problems thinking Pale or clay-colored stools Exams and Tests The health care provider will do a physical exam.The following tests can check to see if your liver is working properly:Albumin blood test Liver function tests (serum alkaline phosphatase is most important) Liver function testsLiver function tests are common tests that are used to see how well the liver is working. Tests include:AlbuminAlpha-1 antitrypsinAlkaline phosphata...ImageRead Article Now Book Mark Article Prothrombin time (PT) PTProthrombin time (PT) is a blood test that measures the time it takes for the liquid portion (plasma) of your blood to clot. A related blood test is ...Read Article Now Book Mark Article Cholesterol and lipoprotein blood tests Other tests that can help measure how severe liver disease may be include:Elevated immunoglobulin M level in the blood Liver biopsy Liver biopsyA liver biopsy is a test that takes a sample of tissue from the liver for examination.ImageRead Article Now Book Mark Article Anti-mitochondrial antibodies (results are positive in about 95% of cases) Anti-mitochondrial antibodiesAntimitochondrial antibodies (AMA) are substances (antibodies) that form against mitochondria. The mitochondria are an important part of cells. The...ImageRead Article Now Book Mark Article Special types of ultrasound or MRI that measure the amount of scar tissue (may be called elastography) Magnetic resonance cholangiopancreatography (MRCP) Treatment The goal of treatment is to ease symptoms and prevent complications.Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream. This may improve survival in some people. A newer drug called obeticholic acid (Ocaliva) is also available.Vitamin replacement therapy restores vitamins A, K, E and D, which are lost in fatty stools. A calcium supplement or other bone medicines may be added to prevent or treat weak or soft bones.Weak or soft bonesOsteoporosis is a disease in which bones become fragile and more likely to break (fracture).ImageRead Article Now Book Mark Article Long-term monitoring and treatment of liver failure is needed.Monitoring and treatment of liver failu...Cirrhosis is scarring of the liver and poor liver function. It is the last stage of chronic liver disease. You were in the hospital to treat this c...Read Article Now Book Mark Article Liver transplant may be successful if it is done before liver failure occurs. Liver transplantLiver transplant is surgery to replace a diseased liver with a healthy liver.ImageRead Article Now Book Mark Article Outlook (Prognosis) The outcome can vary. If the condition is not treated, most people will die without a liver transplant. About one quarter of people who have had the disease for 10 years will have liver failure. Doctors can now use a statistical model to predict the best time to do the transplant. Other diseases, such as hypothyroidism and anemia, can also develop. Possible Complications Progressive cirrhosis can lead to liver failure. Complications can include:Bleeding Damage to the brain (encephalopathy) EncephalopathyLoss of brain function occurs when the liver is unable to remove toxins from the blood. This is called hepatic encephalopathy (HE). This problem ma...Read Article Now Book Mark Article Fluid and electrolyte imbalance ElectrolyteElectrolytes are minerals in your blood and other body fluids that carry an electric charge. Electrolytes affect how your body functions in many ways...Read Article Now Book Mark Article Kidney failure Malabsorption MalabsorptionMalabsorption involves problems with the body's ability to take in (absorb) nutrients from food.ImageRead Article Now Book Mark Article Malnutrition MalnutritionMalnutrition is the condition that occurs when your body does not get enough nutrients.ImageRead Article Now Book Mark Article Soft or weak bones (osteomalacia or osteoporosis) Ascites (fluid buildup in the abdominal cavity) Increased risk of liver cancer When to Contact a Medical Professional Call your provider if you have:Abdominal swelling Blood in the stools Blood in the stoolsBlack or tarry stools with a foul smell are a sign of a problem in the upper digestive tract. It most often indicates that there is bleeding in the ...Read Article Now Book Mark Article Confusion Jaundice Itching of the skin that does not go away and is not related to other causes Vomiting bloodVomiting bloodVomiting blood is regurgitating (throwing up) contents of the stomach that contains blood. Vomited blood may appear bright red, dark red, or look lik...Read Article Now Book Mark Article Open ReferencesReferencesEaton JE, Lindor KD. Primary biliary cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 91.Fogel EL, Sherman S. Diseases of the gallbladder and bile ducts. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 146.Lamps LW. Liver: non-neoplastic diseases. In: Goldblum JR, Lamps LW, McKenney JK, Myers JL, eds. Rosai and Ackerman's Surgical Pathology. 11th ed. Philadelphia, PA: Elsevier; 2018:chap 19.Smith A, Baumgartner K, Bositis C. Cirrhosis: diagnosis and management. Am Fam Physician. 2019;100(12):759-770. PMID: 31845776 pubmed.ncbi.nlm.nih.gov/31845776/.AllVideoImagesTogDigestive system - illustration The esophagus, stomach, large and small intestine, aided by the liver, gallbladder and pancreas convert the nutritive components of food into energy and break down the non-nutritive components into waste to be excreted.Digestive systemillustrationBile pathway - illustration The biliary system is comprised of the organs and duct system that create, transport, store and release bile into the duodenum for digestion. Includes the liver, gallbladder and bile ducts (named the cystic, hepatic, common, and pancreatic duct).Bile pathwayillustrationDigestive system - illustration The esophagus, stomach, large and small intestine, aided by the liver, gallbladder and pancreas convert the nutritive components of food into energy and break down the non-nutritive components into waste to be excreted.Digestive systemillustrationBile pathway - illustration The biliary system is comprised of the organs and duct system that create, transport, store and release bile into the duodenum for digestion. Includes the liver, gallbladder and bile ducts (named the cystic, hepatic, common, and pancreatic duct).Bile pathwayillustrationA Closer Look Cirrhosis(In-Depth)Cirrhosis(Alt. Medicine) Tests for Primary biliary cirrhosis Antimitochondrial antibodyAutoimmune liver disease panelRelated Information Bile(Special Topic)Cirrhosis(Condition)Autoimmune disorders(Condition)Cirrhosis - discharge(Discharge)Cirrhosis(In-Depth) Review Date: 4/30/2020 Reviewed By: Bradley J. Winston, MD, board certified in gastroenterology and hepatology, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- © 1997- All rights reserved. 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