Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis (PSC) is a rare, progressive and chronic liver disease characterized by inflammation and scarring of the bile ducts, which are the small tubes that carry bile from the liver to the small intestine. PSC can lead to blockage of the bile ducts and damage to the liver.


The cause of PSC is not well understood, but it is believed to be an autoimmune disorder.  PSC can lead to serious complications such as liver failure, which may require a liver transplant. PSC is often associated with inflammatory bowel disease (IBD), particularly ulcerative colitis.


  • Jaundice (yellowing of the skin and eyes).
  • Abdominal pain.
  • Fever, chills and night sweats.
  • Itching.
  • Fatigue.
  • Weight loss.
  • Diarrhea.


Diagnosis of PSC typically involves a combination of blood tests, imaging (via radiology and/or endoscopically) tests, and rarely a liver biopsy.


Primary sclerosing cholangitis (PSC) is a chronic liver disease that can lead to a number of complications. Some of the most common complications of PSC include:

  • Cirrhosis and Portal hypertension. In PSC, the progressive inflammation and obstruction of the bile ducts leads to the development of fibrosis and cirrhosis which in turn leads to portal hypertension. Portal hypertension is a condition in which the pressure in the portal vein, which carries blood from the gastrointestinal tract to the liver, becomes elevated. This can occur as a result of cirrhosis, and can lead to a number of complications such as variceal bleeding, ascites, and hepatic encephalopathy.
  • Scarring of the bile duct leads to biliary obstruction, which can lead to bile stasis and bacterial overgrowth in the biliary tree. Risk of infection is high after endoscopic interventions to stretch open the bile duct narrowing and stone removal from the bile duct. 
  • Primary sclerosing cholangitis increases the risk of developing bile duct cancer, also known as cholangiocarcinoma. Bile duct cancer is a rare but serious complication of PSC.  PSC can also increase the risk of gallbladder cancer and hepatocellular carcinoma.
  • Primary sclerosing cholangitis increases the risk of developing colon cancer in patients with inflammatory bowel disease (IBD). People with PSC/IBD have a higher risk of colon cancer than the general population and require close endoscopic surveillance.
  • Bile stasis: PSC can cause blockages in the bile ducts, leading to a buildup of bile in the liver and a condition called cholestasis. Symptoms of cholestasis include itching, jaundice (yellowing of the skin and eyes), and fatigue.
  • Bone loss. Primary sclerosing cholangitis can lead to bone loss, also known as osteopenia or osteoporosis. Osteoporosis is a condition in which the bones become weak and brittle, making them more likely to fracture. Management of bone loss in PSC may include adequate intake of vitamin D, calcium and other bone-supportive nutrients, and medications such as bisphosphonates.


  • Ursodeoxycholic acid (UDCA) to reduce inflammation and improve bile flow.
  • Vitamin D and calcium supplement in patients at risk for bone loss.
  • Antibiotics to treat any infection caused by the bile duct obstruction.
  • Management of any underlying conditions such as IBD.
  • In advanced cases, liver transplantation may be necessary.


The exact cause of PSC is not known, but it is believed to be an autoimmune disorder in which the body’s immune system mistakenly attacks the bile ducts. Other possible causes include genetic factors, viral infections, and environmental toxins.

Symptoms of PSC can vary from person to person, but common symptoms include itching, jaundice, fatigue, abdominal pain, and weight loss.

PSC is usually diagnosed based on a combination of symptoms, physical examination, blood tests, imaging tests, and rarely a liver biopsy.

Treatment for PSC may include medications to control symptoms, endoscopy to remove blockages in the bile ducts, and liver transplantation in cases of advanced disease.

There is currently no cure for PSC, but treatment can help to control symptoms and slow the progression of the disease. In some cases, a liver transplant may be necessary to treat advanced PSC.

The exact cause of PSC is not known, so it is not clear how to prevent it. However, maintaining a healthy lifestyle and avoiding certain risk factors, such as viral infections and environmental toxins, may help to reduce the risk of developing PSC.

Long-term complications of PSC can include liver failure, cirrhosis, bile duct cancer, gallbladder cancer, hepatocellular carcinoma, colon cancer, frequent infections, bone loss, and portal hypertension. It is important for people with PSC to be regularly monitored by a specialist to detect and manage any complications that may arise.

While a healthy diet and certain supplements may help to support liver function and overall health, there is currently no specific diet or supplement that has been proven to treat PSC. However, adequate intake of certain nutrients such as vitamin D and calcium may be important to prevent bone loss.

PSC is not a hereditary disease, but there is a slight increased risk if there is a family history of the disease.