BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuSclerosing cholangitisPrimary sclerosing cholangitis; PSC Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.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 Causes The cause of this condition is unknown in most cases.The disease may be seen in people who have:Inflammatory bowel disease (IBD) such as ulcerative colitis and Crohn disease Ulcerative colitisUlcerative colitis is a condition in which the lining of the large intestine (colon) and rectum become inflamed. It is a form of inflammatory bowel ...ImageRead Article Now Book Mark Article Crohn diseaseCrohn disease is a disease where parts of the digestive tract become inflamed. It most often involves the lower end of the small intestine and the be...ImageRead Article Now Book Mark Article Autoimmune disorders Autoimmune disordersAn autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...ImageRead Article Now Book Mark Article Chronic pancreatitis (inflamed pancreas) Chronic pancreatitisPancreatitis is swelling of the pancreas. Chronic pancreatitis is present when this problem does not heal or recurs and does not improve, gets worse...ImageRead Article Now Book Mark Article Sarcoidosis (a disease that causes inflammation in various parts of the body)SarcoidosisSarcoidosis is a disease in which inflammation occurs in the lymph nodes, lungs, liver, eyes, skin, and/or other tissues.ImageRead Article Now Book Mark Article Genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.Sclerosing cholangitis may also be caused by:Choledocholithiasis (gallstones in the bile duct) CholedocholithiasisCholedocholithiasis is the presence of at least one gallstone in the common bile duct. The stone may be made up of bile pigments or calcium and chol...ImageRead Article Now Book Mark Article Infections in the liver, gallbladder, and bile ducts Symptoms The first symptoms are usually:Fatigue Itching Yellowing of the skin and eyes (jaundice) However, some people have no symptoms.Other symptoms may include:Enlarged liver Enlarged liverEnlarged liver refers to swelling of the liver beyond its normal size. Hepatomegaly is another word to describe this problem. If both the liver and ...ImageRead Article Now Book Mark Article Enlarged spleen Enlarged spleenSplenomegaly is a larger-than-normal spleen. The spleen is an organ in the upper left part of the belly.ImageRead Article Now Book Mark Article Loss of appetite and weight loss Repeat episodes of cholangitis (an infection of the bile duct that causes fever, jaundice and pain in the right upper abdomen)CholangitisCholangitis is an infection of the bile ducts, the tubes that carry bile from the liver to the gallbladder and intestines. Bile is a liquid made by ...ImageRead Article Now Book Mark Article Exams and Tests Even though some people do not have symptoms, blood tests shows that they have abnormal liver function. Your health care provider will look for:Diseases that cause similar problems Diseases that often occur with this condition (especially IBD) GallstonesTests that show cholangitis include:Abdominal CT scan Abdominal CT scanAn abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...ImageRead Article Now Book Mark Article Abdominal ultrasound Abdominal ultrasoundAbdominal ultrasound is a type of imaging test. It is used to look at organs in the abdomen, including the liver, gallbladder, spleen, pancreas, and...ImageRead Article Now Book Mark Article Endoscopic retrograde cholangiopancreatography (ERCP) Endoscopic retrograde cholangiopancreat...ERCP is short for endoscopic retrograde cholangiopancreatography. It is a procedure that looks at the bile and pancreatic ducts. It is done through...ImageRead Article Now Book Mark Article 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 Magnetic resonance cholangiopancreatography (MRCP) Percutaneous transhepatic cholangiogram (PTC) PTCA percutaneous transhepatic cholangiogram (PTC) is an x-ray of the bile ducts. These are the tubes that carry bile from the liver to the gallbladder...ImageRead Article Now Book Mark Article Blood tests for liver enzymes (liver function tests)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 Treatment Medicines that may be used include:Cholestyramine (such as Prevalite) to treat itching Ursodeoxycholic acid (ursodiol) to improve liver function Fat-soluble vitamins (D, E, A, K) to replace what is lost from the disease itself Antibiotics to treat infections in the bile ductsThese surgical procedures may be done:Inserting a long, thin tube with a balloon at the end to open up narrowing (endoscopic balloon dilation of strictures) EndoscopicAn endoscope is a medical device with a light attached. It is used to look inside a body cavity or organ. The scope is inserted through a natural o...Read Article Now Book Mark Article Placement of a drain or tube for major narrowing (strictures) of bile ducts Proctocolectomy (removal of colon and rectum, for those who have both ulcerative colitis and sclerosing cholangitis) does not affect the progression of primary sclerosing cholangitis (PSC) ProctocolectomyTotal proctocolectomy with ileostomy is surgery to remove all of the colon (large intestine) and rectum.Read Article Now Book Mark Article Liver transplant Outlook (Prognosis) How well people do varies. The disease tends to get worse over time. Sometimes people develop:Ascites (buildup of fluid in the space between the lining of the abdomen and abdominal organs) and varices in the esophagus or stomach (enlarged veins) AscitesAscites is the build-up of fluid in the space between the lining of the abdomen and abdominal organs.ImageRead Article Now Book Mark Article VaricesThe esophagus (food pipe) is the tube that connects your throat to your stomach. Varices are enlarged veins that may be found in the esophagus in pe...ImageRead Article Now Book Mark Article Biliary cirrhosis (liver scarring due to inflammation of the bile ducts) CirrhosisCirrhosis is scarring of the liver and poor liver function. It is the last stage of chronic liver disease.ImageRead Article Now Book Mark Article Liver failure Persistent jaundiceSome people develop infections of the bile ducts that keep returning.People with this condition have a high risk for developing cancer of the bile ducts (cholangiocarcinoma). They should be checked regularly with a liver imaging test and blood tests. People who also have IBD may have an increased risk for developing cancer of the colon or rectum and should have periodic colonoscopy. Possible Complications Complications may include:Bleeding esophageal varices Bleeding esophageal varicesThe esophagus (food pipe) is the tube that connects your throat to your stomach. Varices are enlarged veins that may be found in the esophagus in pe...ImageRead Article Now Book Mark Article Cancer in the bile ducts (cholangiocarcinoma) CholangiocarcinomaCholangiocarcinoma (CCA) is a rare cancerous (malignant) growth in one of the ducts that carries bile from the liver to the small intestine.ImageRead Article Now Book Mark Article Cirrhosis and liver failure CirrhosisCirrhosis is scarring of the liver and poor liver function. It is the last stage of chronic liver disease.ImageRead Article Now Book Mark Article Infection of the biliary system (cholangitis) CholangitisCholangitis is an infection of the bile ducts, the tubes that carry bile from the liver to the gallbladder and intestines. Bile is a liquid made by ...ImageRead Article Now Book Mark Article Narrowing of the bile ducts Vitamin deficiencies Open ReferencesReferencesAhrendt GM, Ahrendt SA. Gallbladder and biliary tree: Management of primary sclerosing cholangitis. In: Cameron AM, Cameron JL, eds. Current Surgical Therapy. 14th ed. Philadelphia, PA: Elsevier; 2023:457-538.Bowlus C, Assis DN, Goldberg D. Primary and secondary sclerosing cholangitis. In: Sanyal AJ, Boyer TD, Lindor KD, Terrault NA, eds. Zakim and Boyer's Hepatology. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 43.Levy C, Bowlus CL. Primary and secondary sclerosing cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 68.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 pathwayillustrationRelated Information Bile(Special Topic)Crohn disease(Condition)Cirrhosis(Condition)Biliary system(Special Topic)Crohn disease - InDepth(In-Depth)Cirrhosis - InDepth(In-Depth)Hepatitis - InDepth(In-Depth) Review Date: 5/2/2023 Reviewed By: Michael M. Phillips, MD, Emeritus Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David C. Dugdale, MD, 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. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. © 1997- All rights reserved. A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.Content is best viewed in IE9 or above, Firefox and Google Chrome browser.
Sclerosing cholangitisPrimary sclerosing cholangitis; PSC Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.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 Causes The cause of this condition is unknown in most cases.The disease may be seen in people who have:Inflammatory bowel disease (IBD) such as ulcerative colitis and Crohn disease Ulcerative colitisUlcerative colitis is a condition in which the lining of the large intestine (colon) and rectum become inflamed. It is a form of inflammatory bowel ...ImageRead Article Now Book Mark Article Crohn diseaseCrohn disease is a disease where parts of the digestive tract become inflamed. It most often involves the lower end of the small intestine and the be...ImageRead Article Now Book Mark Article Autoimmune disorders Autoimmune disordersAn autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...ImageRead Article Now Book Mark Article Chronic pancreatitis (inflamed pancreas) Chronic pancreatitisPancreatitis is swelling of the pancreas. Chronic pancreatitis is present when this problem does not heal or recurs and does not improve, gets worse...ImageRead Article Now Book Mark Article Sarcoidosis (a disease that causes inflammation in various parts of the body)SarcoidosisSarcoidosis is a disease in which inflammation occurs in the lymph nodes, lungs, liver, eyes, skin, and/or other tissues.ImageRead Article Now Book Mark Article Genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.Sclerosing cholangitis may also be caused by:Choledocholithiasis (gallstones in the bile duct) CholedocholithiasisCholedocholithiasis is the presence of at least one gallstone in the common bile duct. The stone may be made up of bile pigments or calcium and chol...ImageRead Article Now Book Mark Article Infections in the liver, gallbladder, and bile ducts Symptoms The first symptoms are usually:Fatigue Itching Yellowing of the skin and eyes (jaundice) However, some people have no symptoms.Other symptoms may include:Enlarged liver Enlarged liverEnlarged liver refers to swelling of the liver beyond its normal size. Hepatomegaly is another word to describe this problem. If both the liver and ...ImageRead Article Now Book Mark Article Enlarged spleen Enlarged spleenSplenomegaly is a larger-than-normal spleen. The spleen is an organ in the upper left part of the belly.ImageRead Article Now Book Mark Article Loss of appetite and weight loss Repeat episodes of cholangitis (an infection of the bile duct that causes fever, jaundice and pain in the right upper abdomen)CholangitisCholangitis is an infection of the bile ducts, the tubes that carry bile from the liver to the gallbladder and intestines. Bile is a liquid made by ...ImageRead Article Now Book Mark Article Exams and Tests Even though some people do not have symptoms, blood tests shows that they have abnormal liver function. Your health care provider will look for:Diseases that cause similar problems Diseases that often occur with this condition (especially IBD) GallstonesTests that show cholangitis include:Abdominal CT scan Abdominal CT scanAn abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...ImageRead Article Now Book Mark Article Abdominal ultrasound Abdominal ultrasoundAbdominal ultrasound is a type of imaging test. It is used to look at organs in the abdomen, including the liver, gallbladder, spleen, pancreas, and...ImageRead Article Now Book Mark Article Endoscopic retrograde cholangiopancreatography (ERCP) Endoscopic retrograde cholangiopancreat...ERCP is short for endoscopic retrograde cholangiopancreatography. It is a procedure that looks at the bile and pancreatic ducts. It is done through...ImageRead Article Now Book Mark Article 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 Magnetic resonance cholangiopancreatography (MRCP) Percutaneous transhepatic cholangiogram (PTC) PTCA percutaneous transhepatic cholangiogram (PTC) is an x-ray of the bile ducts. These are the tubes that carry bile from the liver to the gallbladder...ImageRead Article Now Book Mark Article Blood tests for liver enzymes (liver function tests)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 Treatment Medicines that may be used include:Cholestyramine (such as Prevalite) to treat itching Ursodeoxycholic acid (ursodiol) to improve liver function Fat-soluble vitamins (D, E, A, K) to replace what is lost from the disease itself Antibiotics to treat infections in the bile ductsThese surgical procedures may be done:Inserting a long, thin tube with a balloon at the end to open up narrowing (endoscopic balloon dilation of strictures) EndoscopicAn endoscope is a medical device with a light attached. It is used to look inside a body cavity or organ. The scope is inserted through a natural o...Read Article Now Book Mark Article Placement of a drain or tube for major narrowing (strictures) of bile ducts Proctocolectomy (removal of colon and rectum, for those who have both ulcerative colitis and sclerosing cholangitis) does not affect the progression of primary sclerosing cholangitis (PSC) ProctocolectomyTotal proctocolectomy with ileostomy is surgery to remove all of the colon (large intestine) and rectum.Read Article Now Book Mark Article Liver transplant Outlook (Prognosis) How well people do varies. The disease tends to get worse over time. Sometimes people develop:Ascites (buildup of fluid in the space between the lining of the abdomen and abdominal organs) and varices in the esophagus or stomach (enlarged veins) AscitesAscites is the build-up of fluid in the space between the lining of the abdomen and abdominal organs.ImageRead Article Now Book Mark Article VaricesThe esophagus (food pipe) is the tube that connects your throat to your stomach. Varices are enlarged veins that may be found in the esophagus in pe...ImageRead Article Now Book Mark Article Biliary cirrhosis (liver scarring due to inflammation of the bile ducts) CirrhosisCirrhosis is scarring of the liver and poor liver function. It is the last stage of chronic liver disease.ImageRead Article Now Book Mark Article Liver failure Persistent jaundiceSome people develop infections of the bile ducts that keep returning.People with this condition have a high risk for developing cancer of the bile ducts (cholangiocarcinoma). They should be checked regularly with a liver imaging test and blood tests. People who also have IBD may have an increased risk for developing cancer of the colon or rectum and should have periodic colonoscopy. Possible Complications Complications may include:Bleeding esophageal varices Bleeding esophageal varicesThe esophagus (food pipe) is the tube that connects your throat to your stomach. Varices are enlarged veins that may be found in the esophagus in pe...ImageRead Article Now Book Mark Article Cancer in the bile ducts (cholangiocarcinoma) CholangiocarcinomaCholangiocarcinoma (CCA) is a rare cancerous (malignant) growth in one of the ducts that carries bile from the liver to the small intestine.ImageRead Article Now Book Mark Article Cirrhosis and liver failure CirrhosisCirrhosis is scarring of the liver and poor liver function. It is the last stage of chronic liver disease.ImageRead Article Now Book Mark Article Infection of the biliary system (cholangitis) CholangitisCholangitis is an infection of the bile ducts, the tubes that carry bile from the liver to the gallbladder and intestines. Bile is a liquid made by ...ImageRead Article Now Book Mark Article Narrowing of the bile ducts Vitamin deficiencies Open ReferencesReferencesAhrendt GM, Ahrendt SA. Gallbladder and biliary tree: Management of primary sclerosing cholangitis. In: Cameron AM, Cameron JL, eds. Current Surgical Therapy. 14th ed. Philadelphia, PA: Elsevier; 2023:457-538.Bowlus C, Assis DN, Goldberg D. Primary and secondary sclerosing cholangitis. In: Sanyal AJ, Boyer TD, Lindor KD, Terrault NA, eds. Zakim and Boyer's Hepatology. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 43.Levy C, Bowlus CL. Primary and secondary sclerosing cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 68.