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Primary sclerosing cholangitis
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Primary sclerosing cholangitis

Primary sclerosing cholangitis is a chronic liver disease characterized by inflammation and scarring of the bile ducts, leading to bile buildup in the liver, progressive liver damage, and an increased risk of other autoimmune conditions and cancer, likely due to a combination of genetic and environmental factors.

Overview

Primary sclerosing cholangitis is a condition that affects the bile ducts. These ducts carry bile (a fluid that helps to digest fats) from the liver, where bile is produced, to the gallbladder, where it is stored, and to the small intestine, where it aids in digestion. Primary sclerosing cholangitis occurs because of inflammation in the bile ducts (cholangitis) that leads to scarring (sclerosis) and narrowing of the ducts. As a result, bile cannot be released to the gallbladder and small intestine, and it builds up in the liver.

Primary sclerosing cholangitis is usually diagnosed around age 40, and for unknown reasons, it affects men twice as often as women. Many people have no signs or symptoms of the condition when they are diagnosed, but routine blood tests reveal liver problems. When apparent, the earliest signs and symptoms of primary sclerosing cholangitis include extreme tiredness (fatigue), discomfort in the abdomen, and severe itchiness (pruritus). As the condition worsens, affected individuals may develop yellowing of the skin and whites of the eyes (jaundice) and an enlarged spleen (splenomegaly). Eventually, the buildup of bile damages the liver cells, causing chronic liver disease (cirrhosis) and liver failure. Without bile available to digest them, fats pass through the body. As a result, weight loss and shortages of vitamins that are absorbed with and stored in fats (fat-soluble vitamins) can occur. A fat-soluble vitamin called vitamin D helps absorb calcium and helps bones harden, and lack of this vitamin can cause thinning of the bones (osteoporosis) in people with primary sclerosing cholangitis.

Primary sclerosing cholangitis is often associated with another condition called inflammatory bowel disease, which is characterized by inflammation of the intestines that causes open sores (ulcers) in the intestines and abdominal pain. However, the reason for this link is unclear. Approximately 70 percent of people with primary sclerosing cholangitis have inflammatory bowel disease, most commonly a form of the condition known as ulcerative colitis. In addition, people with primary sclerosing cholangitis are more likely to have an autoimmune disorder, such as type 1 diabetes, celiac disease, or thyroid disease, than people without the condition. Autoimmune disorders occur when the immune system malfunctions and attacks the body's tissues and organs. People with primary sclerosing cholangitis also have an increased risk of developing cancer, particularly cancer of the bile ducts (cholangiocarcinoma).

Frequency

An estimated 1 in 10,000 people have primary sclerosing cholangitis, and the condition is diagnosed in approximately 1 in 100,000 people per year worldwide.

Causes & Risk Factors

Primary sclerosing cholangitis is thought to arise from a combination of genetic and environmental factors. Researchers believe that genetic changes play a role in this condition because it often occurs in several members of a family and because immediate family members of someone with primary sclerosing cholangitis have an increased risk of developing the condition. It is likely that specific genetic variations increase a person's risk of developing primary sclerosing cholangitis, and then exposure to certain environmental factors triggers the disorder. However, the genetic changes that increase susceptibility and the environmental triggers remain unclear.

There is evidence that variations in certain genes involved in immune function influence the risk of developing primary sclerosing cholangitis. The most commonly associated genes belong to a family of genes called the HLA complex. The HLA complex helps the immune system distinguish the body's own proteins from proteins made by foreign invaders (such as viruses and bacteria). Each HLA gene has many different normal variations, allowing each person's immune system to react to a wide range of foreign proteins. Specific variations of several HLA genes seem to be present more often in people with primary sclerosing cholangitis than in people who do not have the disorder. These variations may dysregulate the body's immune response, leading to the inflammation of the bile ducts in people with primary sclerosing cholangitis. However, the mechanism is not well understood. Researchers are also studying variations in other genes related to the body's immune function to understand how they contribute to the risk of developing this condition.

Inheritance

The inheritance pattern of primary sclerosing cholangitis is unknown because many genetic and environmental factors are likely to be involved. This condition tends to cluster in families, however, and having an affected family member is a risk factor for developing the disease.

Other Names

  • PSC
  • Sclerosing cholangitis

Frequently Asked Questions

Over time, PSC can lead to serious complications including chronic liver disease (cirrhosis), liver failure, and an increased risk of certain cancers, particularly cholangiocarcinoma (bile duct cancer) and colon cancer (if inflammatory bowel disease is also present). Regular monitoring typically involves blood tests to check liver function, imaging studies like MRI cholangiopancreatography (MRCP) to assess bile ducts, and sometimes liver biopsies. Due to the increased cancer risk, surveillance for cholangiocarcinoma often includes annual MRCPs and blood tests for tumor markers, and colonoscopies may be recommended if you also have inflammatory bowel disease.
While there's no specific diet that cures PSC, a healthy, balanced diet low in processed foods and saturated fats can support overall liver health. For severe itchiness (pruritus), medications like cholestyramine or rifampin may be prescribed. Managing fatigue often involves prioritizing rest, regular light exercise, and avoiding alcohol, which can further damage the liver. Some individuals may benefit from vitamin supplements, especially fat-soluble vitamins (A, D, E, K), as bile duct issues can impair their absorption.
While many individuals are diagnosed incidentally through routine blood tests showing liver abnormalities, the earliest noticeable symptoms of PSC can include extreme tiredness (fatigue), a dull discomfort or pain in the upper right abdomen, and severe, persistent itchiness (pruritus) without a rash. If these symptoms are present, especially in combination, it warrants a visit to a doctor for further investigation, including liver function tests.
There is currently no cure for PSC, and treatments focus on managing symptoms and slowing disease progression. Medications like ursodeoxycholic acid (UDCA) are sometimes used, though their long-term benefits are debated. Endoscopic procedures can be performed to widen narrowed bile ducts. When PSC progresses to severe liver damage or liver failure, a liver transplant becomes the definitive treatment. Transplantation is typically considered when the liver can no longer perform its essential functions, leading to life-threatening complications.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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