Sclerosing cholangitis
Primary sclerosing cholangitis; PSCSclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.
Bile
Bile 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...
Read Article Now Book Mark ArticleCauses
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 colitis
Ulcerative colitis is a condition in which the lining of the large intestine (colon) and rectum become inflamed. It is a form of inflammatory bowel ...
Read Article Now Book Mark ArticleCrohn disease
Crohn 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...
Read Article Now Book Mark Article - Autoimmune disorders
Autoimmune disorders
An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...
Read Article Now Book Mark Article - Chronic pancreatitis (inflamed pancreas)
Chronic pancreatitis
Pancreatitis is swelling of the pancreas. Chronic pancreatitis is present when this problem does not heal or recurs and does not improve, gets worse...
Read Article Now Book Mark Article - Sarcoidosis (a disease that causes inflammation in various parts of the body)
Sarcoidosis
Sarcoidosis is a disease in which inflammation occurs in the lymph nodes, lungs, liver, eyes, skin, and/or other tissues.
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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)
Choledocholithiasis
Choledocholithiasis 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...
Read 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 liver
Enlarged liver refers to swelling of the liver beyond its normal size. Hepatomegaly is another word to describe this problem. If both the liver and ...
Read Article Now Book Mark Article - Enlarged spleen
Enlarged spleen
Splenomegaly is a larger-than-normal spleen. The spleen is an organ in the upper left part of the belly.
Read 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)
Cholangitis
Cholangitis 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 ...
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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)
- Gallstones
Tests that show cholangitis include:
- Abdominal CT scan
Abdominal CT scan
An 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...
Read Article Now Book Mark Article - Abdominal ultrasound
Abdominal ultrasound
Abdominal ultrasound is a type of imaging test. It is used to look at organs in the abdomen, including the liver, gallbladder, spleen, pancreas, and...
Read 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...
Read Article Now Book Mark Article - Liver biopsy
Liver biopsy
A liver biopsy is a test that takes a sample of tissue from the liver for examination.
Read Article Now Book Mark Article - Magnetic resonance cholangiopancreatography (MRCP)
- Percutaneous transhepatic cholangiogram (PTC)
PTC
A 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...
Read Article Now Book Mark Article - Blood tests for liver enzymes (liver function tests)
Liver function tests
Liver function tests are common tests that are used to see how well the liver is working. Tests include:AlbuminAlpha-1 antitrypsinAlkaline phosphata...
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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 ducts
These 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)
Endoscopic
An 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)
Proctocolectomy
Total 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)
Ascites
Ascites is the build-up of fluid in the space between the lining of the abdomen and abdominal organs.
Read Article Now Book Mark ArticleVarices
The 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...
Read Article Now Book Mark Article - Biliary cirrhosis (liver scarring due to inflammation of the bile ducts)
Cirrhosis
Cirrhosis is scarring of the liver and poor liver function. It is the last stage of chronic liver disease.
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- Persistent jaundice
Some 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 varices
The 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...
Read Article Now Book Mark Article - Cancer in the bile ducts (cholangiocarcinoma)
Cholangiocarcinoma
Cholangiocarcinoma (CCA) is a rare cancerous (malignant) growth in one of the ducts that carries bile from the liver to the small intestine.
Read Article Now Book Mark Article - Cirrhosis and liver failure
Cirrhosis
Cirrhosis is scarring of the liver and poor liver function. It is the last stage of chronic liver disease.
Read Article Now Book Mark Article - Infection of the biliary system (cholangitis)
Cholangitis
Cholangitis 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 ...
Read Article Now Book Mark Article - Narrowing of the bile ducts
- Vitamin deficiencies
References
Ahrendt 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.
Digestive 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 system
illustration
Bile 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 pathway
illustration
Digestive 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 system
illustration
Bile 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 pathway
illustration
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.