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British Society of Gastroenterology and UK-PSC Guidelines for the Diagnosis and Management of Gut: First Published As 10.1136/Gutjnl-2018-317993 on 1 June 2019

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British Society of Gastroenterology and UK-PSC Guidelines for the Diagnosis and Management of Gut: First Published As 10.1136/Gutjnl-2018-317993 on 1 June 2019 Guidelines British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of Gut: first published as 10.1136/gutjnl-2018-317993 on 1 June 2019. Downloaded from primary sclerosing cholangitis Michael Huw Chapman, 1,2 Douglas Thorburn,2 Gideon M Hirschfield,3 George G J Webster,1 Simon M Rushbrook,4 Graeme Alexander,2 Jane Collier,5 Jessica K Dyson,6,7 David EJ Jones,7 Imran Patanwala,8,9 Collette Thain,10 Martine Walmsley,11 Stephen P Pereira1,12 1GI Division, UCL Hospitals NHS ABSTRact cholangiocarcinoma (CCA). Patients with PSC Foundation Trust, London, UK 2 These guidelines on the management of primary should ordinarily not undergo endoscopic retro- Liver Unit, Royal Free London grade cholangiopancreatography (ERCP) until NHS Foundation Trust, London, sclerosing cholangitis (PSC) were commissioned by UK the British Society of Gastroenterology liver section. there has been expert multidisciplinary assessment 3Toronto Centre for Liver The guideline writing committee included medical to justify endoscopic intervention. Colitis should be Disease, University Health representatives from hepatology and gastroenterology sought in all patients with PSC using colonoscopy Network and University of groups as well as patient representatives from PSC and colonic biopsies. Patients with colitis should Toronto, Toronto, Canada 4Department of Hepatology, Support. The guidelines aim to support general then have annual surveillance colonoscopy because Norfolk and Norwich University physicians, gastroenterologists and surgeons in of the increased risk of colorectal cancer. In these Hospitals NHS Trust, Norwich, managing adults with PSC or those presenting with guidelines, we also review the management of PSC UK similar cholangiopathies which may mimic PSC, such as overlap syndromes and IgG4-related sclerosing 5Hepatology, John Radcliffe Hospital, Oxford, UK IgG4 sclerosing cholangitis. It also acts as a reference cholangitis (IgG4-SC). 6Hepatology, Newcastle upon for patients with PSC to help them understand their own Tyne Hospitals NHS Foundation management. Quality of evidence is presented using Trust, Newcastle, UK List of recommendations 7 the AGREE II format. Guidance is meant to be used as a Institute of Cellular Medicine, reference rather than for rigid protocol-based care as we 1. There are multiple causes of cholangiopathy. Newcastle University, We recommend that cholestatic liver biochem- Newcastle, UK understand that management of patients often requires 8Gastroenterology, Royal individual patient-centred considerations. istry with typical cholangiographic features Liverpool and Broadgreen in the absence of other identifiable causes of http://gut.bmj.com/ University Hospitals NHS Trust, secondary sclerosing cholangitis is usually Liverpool, UK 9 sufficient for a diagnosis of PSC (strength University of Liverpool, of recommendation: STRONG; quality of Liverpool, UK EXECUTIVE SUMMARY AND 10PBC Foundation, Edinburgh, RECOMMENDATIONS evidence: MODERATE). UK There are many causes of cholangiopathy and these 2. We recommend that MRCP should be the prin- 11 Chair of Trustees PSC Support, should be considered in the assessment of all patients cipal imaging modality for the investigation of Didcot, UK presenting with biliary strictures. Primary sclerosing suspected PSC. ERCP should be reserved for on September 26, 2021 by guest. Protected copyright. 12Institute for Liver & Digestive Health, University College cholangitis (PSC) has a wide spectrum of symptom- patients with biliary strictures requiring tissue London, London, UK atology and stages of disease. Diagnosis is based acquisition (eg, cytological brushings) or where on the cholangiographic (or histological) features therapeutic intervention is indicated (strength Correspondence to of sclerosing cholangitis in the absence of identifi- of recommendation: STRONG; quality of ev- Prof Stephen P Pereira; able causes of secondary sclerosing cholangitis. The idence: HIGH). stephen. pereira@ ucl. ac. uk diagnosis and management of PSC can be difficult 3. We recommend that liver biopsy is normally Received 25 November 2018 and requires specialist referral for advanced disease reserved for possible small duct PSC, assess- Revised 21 February 2019 or patients experiencing significant clinical events. ment of suspected possible overlap variants Accepted 24 March 2019 Few randomised controlled trials have been carried or instances where the diagnosis is unclear Published Online First out to define best management. Most recommen- (strength of recommendation: STRONG; quali- 1 June 2019 dations derive from small case –control studies, ty of evidence: MODERATE). retrospective series and expert opinion. There is 4. We recommend risk stratification based on little evidence for the use of medical therapy to non-invasive assessment. Clinical scores are prevent progression of disease. Ursodeoxycholic an emerging theme but no single method can © Author(s) (or their acid is not recommended for routine use in newly be recommended at present to predict indi- employer(s)) 2019. Re-use permitted under CC BY-NC. No diagnosed PSC. Non-invasive investigations such vidual patient prognosis. Given the unpre- commercial re-use. See rights as magnetic resonance cholangiopancreatography dictable disease course and the serious nature and permissions. Published (MRCP), dynamic liver MRI and/or contrast CT of the complications of PSC, patients should by BMJ. should be performed in patients who have new receive lifelong follow-up (strength of recom- To cite: Chapman MH, or changing symptoms or evolving abnormali- mendation: STRONG; quality of evidence: Thorburn D, Hirschfield GM, ties in laboratory investigations. Worsening liver VERY LOW). et al. Gut biochemistry and/or new high grade or evolving 5. We recommend that ursodeoxycholic acid 2019;68:1356–1378. strictures should prompt further investigation for (UDCA) is not used for the routine treatment 1356 Chapman MH, et al. Gut 2019;68:1356–1378. doi:10.1136/gutjnl-2018-317993 Guidelines of newly diagnosed PSC (strength of recommendation: 18. PSC is a well-recognised indication for liver transplanta- STRONG; quality of evidence: GOOD). For patients al- tion. We recommend that eligibility and referral should ready established on UDCA therapy, there may be evidence be assessed in line with the national guidelines (strength of Gut: first published as 10.1136/gutjnl-2018-317993 on 1 June 2019. Downloaded from of harm in patients taking high dose UDCA 28–30 mg/kg/ recommendation: STRONG; quality of evidence: HIGH). day (strength of recommendation: WEAK; quality of evi- 19. We recommend that all patients with PSC should have a dence: LOW). risk assessment for osteoporosis. Once osteoporosis is 6. We recommend that UDCA is not used for the prevention of detected, treatment and follow-up should be in accord- colorectal cancer or cholangiocarcinoma (strength of recom- ance with national guidelines (strength of recommendation: mendation: STRONG; quality of evidence: HIGH). STRONG; quality of evidence: MODERATE). 7. We recommend that corticosteroids and immunosuppres- 20. Poor nutrition and fat-soluble vitamin deficiency are rela- sants are not indicated for the treatment of classic PSC tively common in advanced PSC and we suggest that clini- (strength of recommendation: STRONG; quality of evi- cians should have a low threshold for empirical replacement dence: HIGH). In those patients with additional features (strength of recommendation: WEAK; quality of evidence: of autoimmune hepatitis (AIH) or IgG4-related sclerosing MODERATE). cholangitis (IgG4-SC), corticosteroids may be indicated 21. We recommend that in patients with fatigue, alternative (strength of recommendation: STRONG; quality of evi- causes should be actively sought and treated (strength of dence: MODERATE). recommendation: STRONG; quality of evidence: LOW). 8. We recommend that endoscopic screening for oesophageal 22. We suggest that cholestyramine (or similar) is first-line varices should be done in line with international guidelines medical treatment for pruritus. Rifampicin and naltrexone where there is evidence of cirrhosis and/or portal hyper- are second-line treatments (strength of recommendation: tension (strength of recommendation: STRONG; quality of WEAK; quality of evidence: LOW). evidence: HIGH). 23. We suggest that an elevated CA19.9 may support a diag- 9. We recommend that colitis should be sought in all patients nosis of suspected cholangiocarcinoma but has a low diag- with PSC using colonoscopy and colonic biopsies (strength nostic accuracy. Routine measurement of serum CA19.9 is of recommendation: STRONG; quality of evidence: not recommended for surveillance for cholangiocarcinoma MODERATE). in PSC (strength of recommendation: WEAK; quality of 10. We recommend that patients with suspected PSC under- evidence: MODERATE). going ERCP should receive prophylactic antibiotics 24. We recommend that when a diagnosis of cholangiocarci- (strength of recommendation: STRONG; quality of evidence: noma is clinically suspected, referral for specialist multi- MODERATE). disciplinary meeting (MDM) review is essential (strength 11. We recommend that non-invasive investigations such as of recommendation: STRONG; quality of evidence: MRCP, dynamic liver MRI and/or contrast CT should be MODERATE). performed in patients who have new or changing symp- 25. We recommend that where cholangiocarcinoma is toms or evolving abnormalities in laboratory investiga- suspected, contrast-enhanced, cross-sectional
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