PSC Guidelines

Guidelines for the diagnosis and management of PSC

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What are Guidelines?

When medical teams care for patients, they often refer to Guidelines. Guidelines are a set of recommendations 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.

Is there a Guideline for PSC?

Yes. There are several regional guidelines for PSC, so depending on where you live, you might receive slightly different care. There are guidelines for the United States, Europe and the UK. The guidelines are broadly the same, with differences mainly based on individual healthcare systems.

Regional Guidelines for Diagnosing and Managing PSC

Europe

United States

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.

PSC Guidelines in the UK

Professor Douglas Thorburn UKPSC Study Day Guidelines

Professor Douglas Thorburn introduces the UK-PSC Guidelines

The guidelines for the diagnosis and management of PSC in the UK were published in 2019 and officially launched at the UK-PSC Study Day by Professor Douglas Thorburn. They were written by UK-PSC, and are endorsed by the British Society of Gastroenterology.  PSC Support incorporated the guideline recommendations into the 'Your Medical Care' section of our website as soon as they were published. Refer to our Questions for Your Doctor page to help guide your routine PSC hospital appointments. See below for the recommendations made in the guideline.

What are the UK-PSC Guideline Recommendations?

To read the guideline in full, you can download them here. We have prepared a list of the main recommendations in the guideline and what this means for your care and what to expect.

We are very grateful to Professor Douglas Thorburn (Royal Free Hospital, London), Chair of UK-PSC for reviewing this information for PSC Support.

Click on the headings below to find the section you are looking for:

Type UK-PSC Guideline Recommendations and What to Expect Strength Quality of Evidence

1. There are multiple causes of cholangiopathy. We recommend thatcholestatic liver biochemistry with typical cholangiographic features in the absence of other identifiable causes of secondary sclerosing cholangitis is usually sufficient for a diagnosis of PSC.


Expect your doctor to consider and exclude other causes of bile duct disease before diagnosing PSC.

Strong Moderate
Diagnosis 2 . We recommend that MRCP should be the principal imaging modality for the investigation of suspected PSC. ERCP should be reserved for patients with biliary strictures requiring tissue acquisition (eg, cytological brushings) or where therapeutic intervention is indicated.


PSC is usually diagnosed with an MRI scan of your bile ducts called an MRCP. The invasive endoscopy procedure ERCP that allows your doctor to look inside your bile ducts, is reserved for treating a blockage in the bile duct or taking a tissue sample.

Strong High
Diagnosis 3. We recommend that liver biopsy is normally reserved for possible small duct PSC, assessment of suspected possible overlap variants or instances where the diagnosis is unclear.


Liver biopsies are not routinely required to make a diagnosis of PSC and are generally undertaken only if: a PSC diagnosis is unclear; or if a type of PSC that is too small to be seen by MRI scan is suspected (small duct PSC); or if autoimmune hepatitis is suspected.

Strong Moderate

30. We recommend that patients with PSC should be encouraged to participate in patient support groups.


Expect to be signposted to PSC Support or other patient support groups. There may be a great group at your own hospital.

Strong Very low

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.


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.

Strong Very Low
Your care 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.


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.

Weak Low

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.


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.

Strong Low

17. We suggest that patients with PSC meeting inclusion criteria should be offered referral to a centre participating in clinical trials.


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.

Weak Low

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.


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.

Strong Moderate
Symptoms 21. We recommend that in patients with fatigue, alternative causes should be actively sought and treated.


Expect your PSC doctor to ask you about symptoms. If you have fatigue, expect your doctor to investigate the possible cause, because some causes of fatigue are treatable.

 

Strong Low
Symptoms 22. We suggest that cholestyramine (or similar) is first-line medical treatment for pruritus. Rifampicin and naltrexone are second-line treatments.


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.

Weak Low

9. We recommend that colitis should be sought in all patients with PSC using colonoscopy and colonic biopsies.


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.

Strong Moderate
Routine tests 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.


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.

Strong Moderate
Routine tests 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.


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.

Weak Moderate
Routine tests 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.


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.

Weak Moderate
Routine tests 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.


Expect to have a yearly ultrasound scan. Your case will be referred to a specialist team for review if a polyp is found. Note: if you have cirrhosis, ultrasound scans will be more frequent (see recommendation 28 under cancer screening).

Weak Low
Routine tests 28. We suggest that in the presence of cirrhosis, hepatocellular carcinoma surveillance should be carried out in accordance with international guidelines.


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

Weak Low
Routine tests 27. 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


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

Strong High
Routine tests 27b. We suggest that those without IBD may benefit from less frequent 5-year colonoscopy or earlier in the advent of new symptoms.


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.

Weak Very low
Routine tests 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.


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

Strong High

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.


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.

Strong High

5. We recommend that ursodeoxycholic acid (UDCA) is not used for the routine treatment of newly diagnosed 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.

Strong Good
Treatment 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.


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.

Weak Low
Treatment 6. We recommend that UDCA is not used for the prevention of colorectal cancer or cholangiocarcinoma.


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.

Strong High
Treatment 7. We recommend that corticosteroids and immunosuppressants are not indicated for the treatment of classic PSC.


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

Strong High
Treatment 7b. In those patients with additional features of autoimmune hepatitis (AIH) or IgG4-related sclerosing cholangitis (IgG4-SC),corticosteroids may be indicated.


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

Strong Moderate

24. We recommend that when a diagnosis of cholangiocarcinoma is clinically suspected, referral for specialist multidisciplinary meeting (MDM) review is essential.


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.

Strong Moderate
Bile duct cancer 25. We recommend that where cholangiocarcinoma is suspected, contrast-enhanced, cross-sectional imaging remains the initial preferred investigation for diagnosis and staging.


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

Strong High
Bile duct cancer 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.


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.

Strong High

Pregnancy
29. We recommend that because pregnancy in cirrhotic patients carries a higher risk of maternal and foetal complications, patients should have preconception counselling and specialist monitoring.


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.

Strong Low

ERCP
10. We recommend that patients with suspected PSC undergoing ERCP should receive prophylactic antibiotics.


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

Strong Moderate
ERCP 12. We recommend that patients with PSC should ordinarily not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention.


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

Strong Moderate
ERCP 13. We recommend that in patients undergoing ERCP for dominant strictures, pathological sampling of suspicious strictures is mandatory.


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.

Strong Strong
ERCP 14. We recommend that in patients undergoing ERCP for dominant strictures, biliary dilatation is preferred to the insertion of biliary stents.


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.

Strong Moderate

Credit

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

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