Learn About Sclerosing Cholangitis

What is the definition of Sclerosing Cholangitis?

Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.

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What are the alternative names for Sclerosing Cholangitis?

Primary sclerosing cholangitis; PSC

What are the causes of Sclerosing Cholangitis?

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
  • Autoimmune disorders
  • Chronic pancreatitis (inflamed pancreas)
  • Sarcoidosis (a disease that causes inflammation in various parts of the body)

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)
  • Infections in the liver, gallbladder, and bile ducts
What are the symptoms of Sclerosing Cholangitis?

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 spleen
  • Loss of appetite and weight loss
  • Repeat episodes of cholangitis
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What are the current treatments for Sclerosing Cholangitis?

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)
  • 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)
  • Liver transplant
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What is the outlook (prognosis) for Sclerosing Cholangitis?

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 (enlarged veins)
  • Biliary cirrhosis (inflammation of the bile ducts)
  • Liver failure
  • 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.

What are the possible complications of Sclerosing Cholangitis?

Complications may include:

  • Bleeding esophageal varices
  • Cancer in the bile ducts (cholangiocarcinoma)
  • Cirrhosis and liver failure
  • Infection of the biliary system (cholangitis)
  • Narrowing of the bile ducts
  • Vitamin deficiencies
Digestive system
Bile pathway
What are the latest Sclerosing Cholangitis Clinical Trials?
Endoskopiskt Register för Diagnostik Och Behandling

Summary: Observational registry including endoscopic diagnostic and therapeutic interventions in the gastrointestinal tract

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An Adaptive, Multicentre, Phase IIa, Multi-disease Trial Investigating the Safety & Activity of a Single Infusion of Selected Mesenchymal Stromal Cells in the Treatment of Patients With Primary Sclerosing Cholangitis & Autoimmune Hepatitis

Summary: MERLIN is an adaptive, single arm, multi-centre, phase IIa multi-disease clinical trial. It is designed to: i) Determine dose safety of ORBCEL-C™ (selected Mesenchymal stromal cells derived from human umbilical cord) ii) Evaluate treatment activity through assessment of biomarkers (for patients treated at the highest safe dose only (HSD)) This trial will determine the Highest Safe Dose (HSD) that ...

What are the Latest Advances for Sclerosing Cholangitis?
Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review.
Gastrointestinal endoscopy in the diagnosis and treatment of biliary liver disease.
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Effect of endoscopic treatment in patients with secondary sclerosing cholangitis.
Who are the sources who wrote this article ?

Published Date: April 20, 2021
Published By: Michael M. Phillips, MD, Emeritus Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

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.

Goussous N, Cunningham SC. The management of primary sclerosing cholangitis. In: Cameron AM, Cameron JL, eds. Current Surgical Therapy. 13th ed. Philadelphia, PA: Elsevier; 2020:469-473.

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.