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Primary sclerosing cholangitis – causes, symptoms, diagnosis, treatment & pathology

What is Primary Sclerosing Cholangitis (PSC)?

Primary Sclerosing Cholangitis (PSC) is a chronic disease of the bile ducts in the liver which become inflamed and scarred and ultimately gets blocked. As the bile ducts get blocked, it accumulates in the liver and damages the liver cells causing cirrhosis or fibrosis. Liver cirrhosis impairs the liver function and finally totally damages the liver where the patient will need a liver transplant to survive.

PSC advances slowly and patients may have the disease for many years before it is even diagnosed. Most people with PSC ultimately require a liver transplant. PSC can also lead to bile duct cancer due to continuous inflammation and scarring. Constant monitoring with MRIs and endoscopy is absolutely essential to pick up cancer in the early stages.

The causes of PSC still remains unknown. New evidence points to the fact that it could be caused by an unknown bacteria or virus in people who already have a genetic predisposition to the disease.
Early warning signs of PSC include:
  • Itching
  • Fatigue
Symptoms which appear as the disease progresses include:
  • Enlarged liver and spleen
  • Pain the upper right quadrant of the abdomen
  • Loss of appetite
  • Fever
  • Weight loss
  • Jaundice
Progressive PSC which leads to cirrhosis and liver failure presents with the following symptoms:
  • Severe jaundice with yellow eyes and skin
  • Edema or swelling in the ankles
  • Bruising and bleeding
  • Bloating or ascites
  • Disorientation, confusion and memory loss

PSC is diagnosed by the doctor with a medical history, physical exam, blood tests, imaging and biopsy if required.

Medical history
The doctor will check if you have inflammatory bowel disease or ulcerative colitis. The doctor will also need to know if anyone in your family has had a history of liver or biliary disorders. You will also have to let the doctor know if you have an autoimmune disorder like type 1 diabetes, celiac disease or thyroid disorders.

Physical exam
The doctor will closely examine your abdomen and check the sites of the liver and spleen for enlargement, tenderness and pain.

Blood tests
The usual blood tests to check for PSC are complete blood count, liver enzyme levels and alpha fetoprotein (to screen for early signs of cancer). Elevated liver enzyme levels may point towards signs of liver failure.

Imaging tests include an ultrasound of the abdomen and a magnetic resonance cholangiopancreatography (MRCP). An additional CT scan maybe needed in case.

Liver biopsy
Liver biopsy is indicated when the doctor suspects advanced cirrhosis or cancer.

Other tests
Other tests like endoscopy may be done to rule out esophageal varices which can cause portal hypertension

Currently, no specific treatment or cure is available for PSC. Treatment is aimed at preventing complications and enabling independent living. Ultimately a liver transplant may be the final solution.

Doctors may treat the blocked bile ducts with procedures like endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography to open it up and channel bile flow.

If you have an infection and inflammation, doctors will usually prescribe antibiotics and anti-inflammatory agents.

Itching can be usually treated with antihistamines. You may be prescribed antiemetics for nausea and vomiting.

It is best to follow a low-fat diet with plenty of liquids, fruits and vegetables.