Routine care and monitoring

Colon cancer

Everyone with both PSC and IBD should have a total colonoscopy to screen for colon cancer with regular follow up ⁴.

Why? People with PSC and IBD have an increased risk of colon cancer compared to the general population and those with IBD alone ¹⁰. You doctor should follow the ‘high risk’ guidance in the BSG/ACPGBI guidelines for colorectal cancer screening for people with PSC ¹⁰⁷.

Initial assessment: Total colonoscopy with colonic biopsies.

Frequency: People with PSC+IBD should have colonoscopies annually.

Evidence:  BSG/UK-PSC Guidelines Recommendation 27 ⁵⁸, EASL Cholestatic Guideline Section 5.2.1 ⁴, BSG/ACPGBI guidelines for colorectal cancer figure 2 ¹⁰⁷.

See Bowel Cancer

Your Colonoscopy

Gallbladder Cancer Screening

Everyone with PSC should be screened for gallbladder cancer with regular follow-up.*

Why? People with PSC appear to have an increased risk of developing gallbladder polyps. This association is independent of underlying IBD. Gallbladder polyps found in people with PSC may have higher rates of potentially cancerous cells compared to general population ^108,109, even in polyps smaller than 1cm. If polyps are identified, referral to the regional hepatobiliary and pancreatic multidisciplinary meeting or Liver Transplant Unit is essential.

Initial assessment: Ultrasound scan.

Frequency: People with PSC should have an annual ultrasound scan to screen for gallbladder cancer.

Evidence: BSG/UK-PSC Guidelines Recommendation 26 ⁵⁸, EASL Cholestatic Guideline Section 5.2.2 ⁴.

*unless they have had their gallbladder removed already.

See Gallbladder Cancer

Liver Cancer Screening

Everyone with PSC should be screened for liver cancer (HCC) with regular follow-up.

Why? Liver cancer is uncommon in PSC, even in the presence of cirrhosis but it is a possibility ¹⁰⁹.

Initial assessment: Ultrasound scan

Frequency: People with PSC without the presence of cirrhosis should have an ultrasound scan annually. People with PSC with cirrhosis should have an ultrasound every six months in line with international HCC guidelines.

Evidence: BSG/UK-PSC Guidelines Recommendation 28 ⁵⁸, EASL Cholestatic Guideline Section 5.2.2 ⁴.

See Liver Cancer

Bile Duct Cancer Screening

Bile duct cancer (cholangiocarcinoma, CCA) is a serious complication of PSC and difficult to screen for and diagnose.

Why? People with PSC have a higher risk of developing bile duct cancer than the general population. The majority of CCA cases are identified within one to two years of the PSC diagnosis ^110,111.

Initial assessment: Discussion of potential CCA symptoms (sudden upper abdominal pain, elevated CA19-9 blood test, worsening liver blood tests, sudden jaundice ESPECIALLY IN THE FIRST 1-2 YEARS FOLLOWING DIAGNOSIS OF PSC). Where CCA is suspected, referral to the regional hepatobiliary and pancreatic multidisciplinary meeting or Liver Transplant Unit is essential.

Frequency: Raised CA19-9in isolation has weak diagnostic accuracy and is not recommended as part of routine surveillance in the UK. CA19-9 is common in PSC and fluctuates, even in the absence of bile duct cancer. There is little evidence to justify its use for reliable surveillance of CCA. However, CA19-9 monitoring is a personal decision and some people with PSC prefer to have their CA19-9 monitored.  Some PSC experts suggest that a surveillance strategy could be MRCP combined with CA19-9 with a cut-off greater than or equal to 129 U/mL ⁶⁶. The International PSC Study Group is examining surveillance methods in PSC around the world, particularly for bile duct cancer, with the hope that it might show which types may be more superior.

Evidence: BSG/UK-PSC Guidelines Recommendation 23 and 24 ⁵⁸, EASL Cholestatic Guideline Section 5.2.2 ⁴

See Bile Duct Cancer

Everyone with PSC should have a risk assessment for osteoporosis. 

Why? Bone density reduces at 1% per year for people with PSC ⁷¹.

Initial assessment: Discussion about symptoms and a DEXA (DXA) scan.

Frequency: Your doctor should follow current national guidelines for osteoporosis for any treatment or follow-up.

Evidence: BSG/UK-PSC Guidelines Recommendation 19 ⁵⁸, EASL Cholestatic Guideline Section 11.3 ⁴.

See Bone Health

This includes itch, pain, fatigue and depression and anxiety and how to help manage them.

See Symptoms

Poor nutrition and fat-soluble vitamin deficiency are common in people with PSC.

Why? Advanced bile duct disease means that fat soluble vitamins are not absorbed by the body properly ¹¹². People with advanced PSC should be screened for fat-soluble vitamin deficiencies and supplemented accordingly ^113,114. You may have a feeling of ‘unrest’ soon after eating food high in fat: feeling over full after eating, feeling like the food isn’t moving, hearing lots of gurgling sounds, smelly wind, and sometimes even vomiting ¹¹⁴.

It is recommend that the following are considered:

• calcium and vitamin D supplementation in all cholestatic patients as part of the osteoporosis prevention protocol (Section 11.3 Rec 3; Section 11.4 Rec 1 ⁴)
• a supplement vitamins A, E and K enterally in the presence of steatorrhoea (fatty stools) or laboratory evidence of malabsorption (Section 11.4 Rec 2 ⁴)
• vitamin K in overt cholestasis and in the context of bleeding, prior to invasive procedures such as sphincterotomy and/ or stent insertion at ERCP (Section 11.4 Rec 3 ⁴)

Initial assessment: Your doctor will ask you about symptoms relating to fat malabsorption. A blood test can be taken to measure vitamin D, A and E.

Frequency? At each visit to your PSC doctor.

Evidence: BSG/UK-PSC Guidelines Recommendation 20 ⁵⁸, EASL Cholestatic Guidelines Section 11.4 ⁴.

See Eating for Health

Everyone with PSC should be assessed for the presence of inflammatory bowel disease (IBD).

Why? As many as 80% of people with PSC have IBD ^1,10. People with PSC and IBD are at an increased risk of colon cancer and so require adequate colon cancer surveillance ¹⁰.

Initial assessment: Your doctor will ask you about any symptoms you may have relating to IBD, and then should invite you to have a total colonoscopy with colonic biopsies ¹⁰.

Frequency: If no evidence of IBD is found, your doctor will monitor you for the presence of IBD by asking you about possible IBD symptoms at your review appointments, and occasionally will order a colonoscopy, usually every 5 years if there are no IBD symptoms ¹⁰. If you have IBD, your doctor will start routine screening for colon cancer (see below).

Evidence: BSG/UK-PSC Guidelines Recommendation 9 ⁵⁸, EASL Cholestatic Guideline Section 5.2.1 ⁴.

See PSC-IBD

For many years, PSC has been poorly understood, and there have been no treatment options other than liver transplantation. There is still no curative treatment for PSC but researchers are working on it.

Professor David Adams, PSC Support Trustee, talked about PSC research in 2015 and said, ‘We are about to enter a very exciting phase where we are going to have new treatments and for some of those I have real hopes will begin to modify this terrible disease.’ We have seen advances in other related diseases over the last 10-15 years such as inflammatory bowel disease, multiple sclerosis, rheumatoid arthritis, giving hope to those affected. Professor Adams continued, ‘I’m hopeful we are entering a phase where the same will apply to PSC.’

It takes on average 17 years from scientific discovery to a new medicine. However, you may have the opportunity to try one of those new drugs now, if you are eligible to take part in a clinical trial.

Not everyone meets the strict criteria to take part, and every trial has slightly different eligibility rules, but we would like to see all patients meeting inclusion criteria offered referral to a centre participating in clinical trials. New recommendations from BSG/UK-PSC suggest that all patients meeting inclusion criteria should be offered referral to a centre participating in clinical trials (Recommendation 17) ⁵⁸.

Your PSC doctor should be aware of current trials - always ask!

Find Clinical Trials

Your doctor will try to establish how well your liver is functioning and if there are other associated issues to manage. This can be difficult in PSC so doctors use a range of tests such as:

• your MRCP scan to look at the extent of scarring (strictures) in your bile ducts;
• your ultrasound scan to look at the shape of your liver. An abnormal shape can suggest there is scarring in the liver.
• your Fibroscan (if your doctor has access to one) to look at liver stiffness. The level of stiffness is thought to represent the level of scarring in the liver and is more meaningful in PSC if the score is very low (under 7) or high (over 40). Scores in between can be difficult to interpret.
• your blood tests to give an indication of how well your liver is functioning.

All these tests will provide a baseline assessment of your condition and can provide clues about what will happen in the short-term future (that is, over the next few years). Your doctor may use a prognostic scoring system to help assist timing of referral for liver transplant (should it be necessary), referral to a specialist PSC clinic, or for inclusion in research studies. Prognostic scoring is an emerging field of research.

Tests and Procedures in PSC