Primary Sclerosing Cholangitis (PSC)

What is PSC 

Primary sclerosing cholangitis, aka PSC, is a rare condition that affects the bile ducts of the liver. ‘Primary’ means that the cause or trigger is often unknown, ‘sclerosing’ means scarring of bile ducts which may be seen on a scan or liver biopsy, and ‘cholangitis’ means inflammation of the bile ducts. It can cause one isolated narrowing (or stricture) in the bile ducts, or lead to the formation of many strictures within the bile ducts. Bile ducts are like plumbing pipes that transport bile out of the liver via gallbladder to the bowel to help digest your food. When a narrowing progresses, the flow of bile can be reduced which can lead to liver damage, scarring and even cirrhosis. Jaundice, the yellow discoloration of patients' eyes and skin, may occur.

In around 70% of patients with PSC, a coexisting condition called inflammatory bowel disease (IBD) such as ulcerative colitis or Crohn’s disease can be present. 

 

What causes PSC

The exact cause of PSC is unclear. It is unrelated to alcohol consumption. Because of the strong association between PSC and IBD, there may be a common cause for both diseases. In theory, the inflamed colon/bowel could allow toxins or bacteria to enter the bloodstream which then track to the liver and cause inflammation and damage to the bile ducts. PSC affects both sexes, but males are twice as likely to suffer with PSC compared to females. 

 

PSC Symptoms 

Many patients with PSC have no symptoms at all. PSC is occasionally suspected when routine liver blood tests return abnormal results, which is more common in patients with Ulcerative Colitis or Crohn's disease (IBD). Some common symptoms are: 

  • tiredness/fatigue

  • abdominal pain/discomfort in the right upper abdomen 

Later in the disease course, patients can experience:

  • itch 

  • jaundice 

  • fevers/chills can rarely occur, due to infection of the bile ducts 

 

PSC diagnosis 

The diagnosis of PSC is based on results from blood tests and imaging of the liver and bile ducts. Occasionally, a liver biopsy may be needed to clarify the diagnosis and also stage the extent of the damage it has caused to the liver. If the diagnosis is suspected, a referral is sent from your primary doctor (GP, other specialist) to a Gastroenterologist/Hepatologist to guide what further tests are needed, and to follow you up long-term.

 

Treatment for PSC

Currently there is no known cure or specific treatment for PSC. Research is ongoing to find treatments that will improve patient outcomes in the future. Treatment to improve bile flow out of the liver, such as ursodeoxycholic acid (UDCA), or antibiotics such as vancomycin, may be trialled, but there is no definitive treatment at present.

The main focus of care is to alleviate symptoms that can occur as a result of the disease process of PSC. Such treatments are listed below:

  • Colestyramine (aka Questran) or Cholestagel/Colesevelam is a medication that is prescribed to help relieve itching. It works by reducing the uptake of bile acids in your system which can cause severe itching. It is not immediately effective, and can take some days before a benefit is felt. Colestyramine is best absorbed after meals, but can cause some side effects such as bloating or constipation. If you notice ill effects while taking cholestyramine, let your doctor know so other treatments can be discussed.

  • Artificial tears or lozenges can be used to help relieve the symptoms of dry eyes and dry mouth if present.

  • When the flow of bile is significantly reduced, deficiencies of vitamin A, D, E, K can develop, and supplements may be prescribed.

  • Endoscopic dilatation or stretching of the bile ducts is an option for some patients. The placement of a stent inside the bile duct to prevent the narrowing from progressing is also an option for some patients with narrowing in a main bile duct.

  • Liver transplant is the ultimate treatment for PSC patients who go on to develop advanced liver scarring, also known as cirrhosis. Consideration for the timing of liver transplant can also be influenced by the burden of symptoms associated with PSC. As PSC can progress slowly over years, the need for transplantation can be assessed in a carefully planned fashion.

  • Patient with advanced PSC are also at risk of developing bile duct cancer. Screening tests such as regular liver blood tests, liver ultrasound or MRI will be organised by your treating team in order to try to detect early changes and investigate accordingly.

  • Colitis, causing diarrhoea, is common in PSC, so a screening colonoscopy will be organised to investigate for this. Treatment is then decided based on the presence of colitis, its extent and severity.

  • If IBD is present, a colonoscopy is performed annually to screen and detect bowel cancer at an early stage, as there is an increased risk in patients with PSC and IBD.

  • Osteoporosis is associated with PSC. This is diagnosed by a bone density scan called a DEXA scan. If detected, medication such as vitamin D, calcium replacement etc can be prescribed to protect your bones, and to reduce the risk of development of fragility fractures.

 

FAQs

Can PSC be prevented?

  • PSC can’t be prevented as the cause for PSC isn’t clearly known. Early diagnosis is key to treat the symptoms associated with it and to screen for complications that can occur as the disease progresses.

 

What should I eat if I have PSC?

  • PSC is associated with malabsorption of fat soluble vitamins such as vitamins A, E, D, and K. As such, you may need to take supplements to make up for these deficiencies, as indicated. Calcium and vitamin D may also need to be supplemented to reduce your risk of developing osteoporosis. Dietary fats may cause a varying degree of symptoms, and some patients feel better when they restrict their intake of high fat foods. One size does not fit all however, so specialist dietary advice may be needed to give you the best advice for you.

  • If you go on to develop liver cirrhosis, specialist dietary advice is given. See our section on diet and liver disease also: https://www.liverfoundation.ie/diet-in-liver-disease

I feel tired and fatigued, is that common with PSC?

  • Fatigue and tiredness is quite common among patients with PSC. You may need to change the pace of your day according to the burden of your symptoms. Maintaining a healthy lifestyle can help, and regular gentle exercise is also recommended.

 

Is there anything my other doctors or dentist should know?

  • Occasionally there is an increased risk of bleeding when undergoing dental procedures/tooth extractions. This is not the case for everyone with PSC so ask your specialist for more information.

  • Other doctors should be informed about your liver diagnosis as your ability to safely process certain medications such as paracetamol, anti-inflammatories, and other medications may be impaired. Feel free to discuss this with your specialist or pharmacist.

Useful links:

https://britishlivertrust.org.uk/information-and-support/living-with-a-liver-condition/liver-conditions/primary-sclerosing-cholangitis/