PSC Care Guidelines

Guidelines for the diagnosis and management of PSC

What are care guidelines?

When medical teams care for patients, they often refer to 'guidelines'. Guidelines are a set of recommendations on the diagnosis and management of a disease that are made by experts, based on evidence (or expert consensus if there is not enough evidence to support the recommendation). The guidelines are referred to in a number of ways, including: Clinical Practice Guidelines, Practice Guideline and Clinical Guideline.

There are several regional guidelines for PSC, so depending on where you live, you might receive slightly different care. The guidelines are broadly the same, with differences mainly based on individual healthcare systems. The guidelines for the diagnosis and management of PSC in the UK were published in 2019 and officially launched by Professor Douglas Thorburn. They were written by UK-PSC with PSC Support, and are endorsed by the British Society of Gastroenterology. We've set out the main recommendations further down this page.

Get the PSC Support App to get the most out of your routine PSC hospital appointments and find information on what to expect from routine appointments.

PSC Support is currently working with European PSC experts to update the 2009 EASL Clinical Practice Guidelines.

Links to regional PSC Guidelines (English language)

Europe

United States

What are the UK recommendations?

Diagnosing PSC

Treatment

See Treatments for PSC

Is UDCA (ursodeoxycholic acid) recommended for people with PSC?

There is insufficient evidence that UDCA provides a benefit to people with PSC and so it is not recommended as a routine treatment for PSC.

There is evidence that high dose UDCA may be harmful, so if you do take it, expect your doctor to ensure you are not taking a high dose.

There is insufficient evidence to support the claim that UDCA can protect against bowel cancer or bile duct cancer so it is not recommended for cancer prevention in PSC.

Scientific Recommendation 5a. We recommend that ursodeoxycholic acid (UDCA) is not used for the routine treatment of newly diagnosed PSC (strength of recommendation: STRONG; quality of evidence: GOOD).

Scientific Recommendation 5b. For patients already established on UDCA therapy, there may be evidence of harm in patients taking high dose UDCA 28–30 mg/kg/day (strength of recommendation: WEAK; quality of evidence: LOW).

Scientific Recommendation 6. We recommend that UDCA is not used for the prevention of colorectal cancer or cholangiocarcinoma (strength of recommendation: STRONG; quality of evidence: HIGH).

Are corticosteroids or immunosuppressants recommended for people with PSC?

Do not expect corticosteroids or immunosuppressants to be used to treat classic PSC.

If you have features of autoimmune hepatitis (AIH) or IgG4-related sclerosing cholangitis (IgG4-SC), your doctor may prescribe corticosteroids and other immunosuppressants.

Scientific Recommendation 7a. We recommend that corticosteroids and immunosuppressants are not indicated for the treatment of classic PSC (strength of recommendation: STRONG; quality of evidence: HIGH).

Scientific Recommendation 7b. In those patients with additional features of autoimmune hepatitis (AIH) or IgG4-related sclerosing cholangitis (IgG4-SC), corticosteroids may be indicated (strength of recommendation: STRONG; quality of evidence: MODERATE).

Your Medical Team

See Working with your Doctor

Do I need to see a PSC specialist?

If you have symptoms, your disease activity is changing or complications of the disease, expect to be referred for multidisciplinary assessment. This might be to a team with experience and expertise of PSC complications. If you have early, stable disease, then expect your care to be managed in a general clinic, with routine monitoring as per these guidelines.

Scientific Recommendation 16. We recommend that patients with symptomatic, evolving or complex disease should be referred for expert multidisciplinary assessment. Patients with early, stable disease can be managed in general clinics (strength of recommendation: STRONG; quality of evidence: LOW).

Do I need life-long medical care for PSC?

Expect to have life-long medical follow-up for PSC because it can be unpredictable and you should be monitored. At the present time there is no single reliable way to predict whether or not you will get complications. Research is ongoing in this area and there might be predictive tools available in the future.

Scientific Recommendation 4. We recommend risk stratification based on non-invasive assessment. Clinical scores are an emerging theme but no single method can be recommended at present to predict individual patient prognosis. Given the unpredictable disease course and the serious nature of the complications of PSC, patients should receive lifelong follow-up (strength of recommendation: STRONG; quality of evidence: VERY LOW).

Should my PSC doctors communicate to my GP about my PSC care and vice versa?

It is helpful if your care is managed by medical teams who communicate and coordinate the management of your care. This may depend on the way your local health services are configured but attention should be paid to your PSC risk assessment and any symptoms you may have.

Scientific Recommendation 15. We suggest that provision of care should involve a partnership between patients, primary care and hospital-led specialty medicine with consideration made with regard to patient risk assessment, symptom burden and how local services are configured (strength of recommendation: WEAK; quality of evidence: LOW).

What is the process for referring someone for liver transplantation?

Some people with PSC need to have a liver transplant. Expect to be referred for some special tests called 'transplant assessment' should your PSC doctor think you may benefit from a liver transplant. It is usual to err on the side of caution with PSC and make this referral earlier rather than later.

Scientific Recommendation 18. PSC is a well-recognised indication for liver transplantation. We recommend that eligibility and referral should be assessed in line with the national guidelines (strength of recommendation: STRONG; quality of evidence: HIGH).

Symptoms

See Symptom Management and When to Contact Your Doctor

What happens if I have new or changing symptoms?

If you have new or changing symptoms or changes are seen in other tests, expect to have further non-invasive investigations such as MRCP (a type of MRI scan), dynamic liver MRI and/or contrast CT scans.

Scientific Recommendation 11. We recommend that non-invasive investigations such as MRCP, dynamic liver MRI and/or contrast CT should be performed in patients who have new or changing symptoms or evolving abnormalities in laboratory investigations (strength of recommendation: STRONG; quality of evidence: MODERATE).

How is fatigue investigated?

What medicine is available for itch (pruritus)?

If you experience itching, expect to be given medicine to help relieve it. There are alternative medicines that can be tried for itch that act differently in your body, so expect to try different medicines if the first doesn't work for you.

Scientific recommendation 22.We suggest that cholestyramine (or similar) is first-line medical treatment for pruritus. Rifampicin and naltrexone are second-line treatments (strength of recommendation: WEAK; quality of evidence: LOW).

Routine Tests and Monitoring

See Your Medical Care

How often can I expect to have an ultrasound scan?

Expect to have a yearly ultrasound scan. Your case will be referred to a specialist team for review if a polyp is found.

If you have cirrhosis, expect more frequent monitoring to check for liver cancer.

Scientific Recommendation 26. We suggest that an annual ultrasound scan of the gallbladder should be performed in patients with PSC. If polyps are identified, treatment should be directed by specialist hepatopancreaticobiliary (HPB) MDM (strength of recommendation: WEAK; quality of evidence: LOW).

Scientific Recommendation 28. We suggest that in the presence of cirrhosis, hepatocellular carcinoma surveillance should be carried out in accordance with international guidelines (strength of recommendation: WEAK; quality of evidence: LOW).

How often can I expect to have a colonoscopy?

Expect to have a colonoscopy when you are diagnosed with PSC to look for colitis (inflammatory bowel disease) if you don't already have it.

Expect to have a yearly colonoscopy if you have IBD and PSC.

Expect to have a 5 yearly colonoscopy if you have PSC but do not have IBD. If you have PSC and have symptoms that mean IBD is suspected, expect to have a colonoscopy.

Scientific Recommendation 9. We recommend that colitis should be sought in all patients with PSC using colonoscopy and colonic biopsies (strength of recommendation: STRONG; quality of evidence: MODERATE).

Scientific Recommendation 27a. We recommend that patients with PSC who have coexistent colonic inflammatory bowel disease (IBD) should have annual colonoscopic surveillance from the time of diagnosis of colitis in line with the British Society of Gastroenterology (BSG) guidelines (strength of recommendation: STRONG; quality of evidence: HIGH).

Scientific Recommendation 27a. We suggest that those without IBD may benefit from less frequent 5-year colonoscopy or earlier in the advent of new symptoms (strength of recommendation: WEAK; quality of evidence: VERY LOW).

When are ERCPs considered for people with PSC?

It is important that an expert multidisciplinary team assesses your case before you have an invasive ERCP procedure.

If you have an ERCP for dominant strictures (narrowings or blockages in the bile ducts outside the liver), the endoscopist may take tissue samples from any areas of concern.

If you have an ERCP, expect the endoscopist to use biliary dilatation(where the bile ducts are widened by inflating a tiny balloon). In some cases stents (short tubes) will be inserted to try widen the bile duct.

If you are having an ERCP, expect to be given a course of antibiotics to take after the procedure.

Scientific Recommendation 12. We recommend that patients with PSC should ordinarily not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention (strength of recommendation: STRONG; quality of evidence: MODERATE).

Scientific Recommendation 13. We recommend that in patients undergoing ERCP for dominant strictures, pathological sampling of suspicious strictures is mandatory (strength of recommendation: STRONG; quality of evidence: STRONG).

Scientific Recommendation 14. We recommend that in patients undergoing ERCP for dominant strictures, biliary dilatation is preferred to the insertion of biliary stents (strength of recommendation: STRONG; quality of evidence: MODERATE).

Scientific Recommendation 10. We recommend that patients with suspected PSC undergoing ERCP should receive prophylactic antibiotics (strength of recommendation: STRONG; quality of evidence: MODERATE).

Will my bone density be assessed?

Expect to have a risk assessment for osteoporosis. This may include a DXA scan. Your PSC doctor will follow national guidelines to monitor and treat osteoporosis if you have it.

Scientific Recommendation 19. We recommend that all patients with PSC should have a risk assessment for osteoporosis. Once osteoporosis is detected, treatment and follow-up should be in accordance with national guidelines (strength of recommendation: STRONG; quality of evidence: MODERATE).

Is nutrition/vitamin deficiency common in people with advanced PSC?

Expect your PSC doctor to ask you about symptoms relating to fat malabsorption and may test your blood for vitamin levels with a view to replacing them.

Scientific Recommendation 20. Poor nutrition and fat-soluble vitamin deficiency are relatively common in advanced PSC and we suggest that clinicians should have a low threshold for empirical replacement (strength of recommendation: WEAK; quality of evidence: MODERATE).

Is CA19.9 tested in people with PSC?

Some doctors measure CA19.9 in the blood to help look for bile duct cancer. CA19.9 levels fluctuate naturally and there is little evidence to justify its use as a reliable way to look for bile duct cancer and so it is not recommended as a routine test for PSC patients.

Scientific Recommendation 23. We suggest that an elevated CA19.9 may support a diagnosis of suspected cholangiocarcinoma but has a low diagnostic accuracy. Routine measurement of serum CA19.9 is not recommended for surveillance for cholangiocarcinoma in PSC (strength of recommendation: WEAK; quality of evidence: MODERATE).

Will I have tests for oesophageal varices?

If you have cirrhosis (liver damage) and/or portal hypertension, expect to have tests for If you have cirrhosis (liver damage) and/or portal hypertension, expect to have tests for oesophageal varices.

Scientific Recommendation 8. We recommend that endoscopic screening for oesophageal varices should be done in line with international guidelines where there is evidence of cirrhosis and/or portal hypertension (strength of recommendation: STRONG; quality of evidence: HIGH).

What happens if bile duct cancer is suspected?

If your doctor suspects bile duct cancer, or wants to check for it, your case must be reviewed in a specialist multidisciplinary meeting (MDM). This is essential.

If bile duct cancer is being investigated, the specialist multidisciplinary meeting (MDM) may use special imaging techniques and this guideline gives recommendations about those.

The specialist multidisciplinary meeting (MDM) investigating bile duct cancer will consider using various tools and technology to take biopsies (tissue samples) and this guideline gives recommendations about those.

Scientific Recommendation 24. We recommend that when a diagnosis of cholangiocarcinoma is clinically suspected, referral for specialist multidisciplinary meeting (MDM) review is essential (strength of recommendation: STRONG; quality of evidence: MODERATE).

Scientific Recommendation 25a. We recommend that where cholangiocarcinoma is suspected, contrast-enhanced, cross-sectional imaging remains the initial preferred investigation for diagnosis and staging (strength of recommendation: STRONG; quality of evidence: HIGH).

Scientific Recommendation 25b. Confirmatory diagnosis relies on histology with the approach to tissue sampling guided by MDM review. Options include ERCP-guided biliary brush cytology/fluorescence in situ hybridisation (FISH)/endobiliary biopsy/cholangioscopy/endoscopic ultrasound (EUS)-guided biopsy and/or percutaneous biopsy (strength of recommendation: STRONG; quality of evidence: HIGH).

What should happen if I am planning a family?

Speak to your doctor if you are planning to have a family so they can arrange the right care for you. This is especially important if you have cirrhosis.

Scientific Recommendation 29. We recommend that because pregnancy in cirrhotic patients carries a higher risk of maternal and fetal complications, patients should have preconception counselling and specialist monitoring (strength of recommendation: STRONG; quality of evidence: LOW).

Taking Part in Research

Find clinical trials for PSC.

Should I take part in research?

Clinical trials to test new drugs for PSC are becoming increasingly common and may be open to recruit patients. Unfortunately not everyone is suitable to take part. If you are suitable and interested, you can ask to be referred to a hospital conducting the trial with details available through PSC Support or UK-PSC.

Scientific Recommendation 17. We suggest that patients with PSC meeting inclusion criteria should be offered referral to a centre participating in clinical trials (strength of recommendation: WEAK; quality of evidence: LOW).

Support

Find out how we can support you.

Should patients be referred to support groups?

Strength and Quality of Recommendations

What does this mean? Each recommendation is rated by a 'strength of recommendation' and a 'quality of evidence' grading. Because PSC is complex and still poorly understood in some ways, some of the evidence used is 'low quality', and that's often because the evidence is simply not available. When this is the case, the recommendation is based on the majority consensus of expert opinion.

Read or download the complete UK-PSC guidelines for the diagnosis and management of PSC

Huge thanks to Professor Douglas Thorburn for reviewing PSC Support's interpretation of each recommendation. 6 November 2019

Chapman MH, Thorburn D, Hirschfield GM, Webster GG, Rushbrook SM, Alexander G, Collier J, Dyson JK, Jones DE, Patanwala I, Thain C, Walmsley M, Pereira S. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut. 2019 Aug 1;68(8):1356-78.