Guideline sources The following summarized guidelines for the evaluation and management of primary sclerosing cholangitis are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL/ILCA 2023), the European Association for the Study of the Liver (EASL 2022), the American Association for the Study of Liver Diseases (AASLD 2022), the British Society of Gastroenterology (BSG 2019), the American Gastroenterological Association (AGA 2019), the Italian Association for the Study of the Liver (AISF 2016), the American College of Gastroenterology (ACG 2015), and the American Society for Gastrointestinal Endoscopy (ASGE 2015). 1 2 3 4 5 6 7 8 9 10 11 11 12 Definition PSC is a chronic autoimmune liver disease characterized by progressive biliary inflammation and fibrosis, resulting in multifocal biliary strictures. 9 Epidemiology https://web.pathway.md/diseases/recCAfARwQnJIjXTi 1/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway Risk factors include family history, genetic susceptibility (HLA class, I, II, and III loci, IL-2 pathway), environmental factors (exposure to farm animals, contraceptive pills, UTIs, diet), and microbial dysbiosis due to IBD. 11 Pathophysiology The incidence and prevalence of PSC is estimated at 0-0.92 per 100,000 person-years, and 0- 13.6 persons per 100,000 population, respectively. 10 Disease course Injury to cholangiocytes leads to biliary inflammation and fibrosis, which may further progress to chronic cholestasis, liver cirrhosis, bile duct strictures, bacterial cholangitis, metabolic bone disease, colon cancer, and cholangiocarcinoma. 12 Prognosis and risk of recurrence PSC is associated with increased risk of cholangiocarcinoma (400 times) and colon cancer (10 times) as compared to the general population. 11 Guidelines 1. Screening and diagnosis Diagnosis: As per EASL 2022 guidelines, diagnose large-duct PSC in adult patients presenting with elevated serum markers of cholestasis in the presence of typical findings of sclerosing cholangitis on high-quality cholangiography (preferably with MRCP) and after exclusion of secondary causes. B Show 2 more As per BSG 2019 guidelines, consider diagnosing PSC based on cholestatic liver biochemistry with typical cholangiographic features in the absence of other identifiable causes of secondary sclerosing cholangitis. B Differential diagnosis, IgG4-related sclerosing cholangitis: As per AASLD 2022 guidelines, measure serum IgG4 levels to exclude IgG4-sclerosing cholangitis in all patients with possible PSC. E As per BSG 2019 guidelines: Do not rely solely on elevated IgG4 levels for distinguishing IgG4-related sclerosing cholangitis from PSC, although it supports the diagnosis of clinically suspected IgG4- related disease. D Recognize that other organ involvement, particularly pancreatic manifestations of IgG4- related disease, may provide important information to distinguish IgG4-related sclerosing cholangitis from PSC. B Differential diagnosis, cholangiocarcinoma: As per EASL 2022 guidelines, suspect cholangiocarcinoma in patients with newly diagnosed PSC with high-grade strictures or with known PSC with worsening of signs or symptoms, https://web.pathway.md/diseases/recCAfARwQnJIjXTi 2/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway progressive strictures, or a new mass lesion identified on imaging. B Show 5 more As per BSG 2019 guidelines, consider measuring CA 19-9 levels to support the diagnosis of suspected cholangiocarcinoma, although recognizing that it has low diagnostic accuracy. C 2. Classification and risk stratification Risk stratification: As per AASLD 2022 guidelines, obtain risk stratification and fibrosis staging at diagnosis of PSC and regularly during follow-up. Consider using clinical risk tools for this purpose but interpret specific probabilities of events with caution in individual patients. E Show 2 more As per EASL 2022 guidelines: Obtain risk assessment at the time of diagnosis and sequentially, based on phenotypic factors and noninvasive tests including: standard biochemistry (including serum bilirubin, albumin, ALP, ALT, platelets, PT) MRI of the liver with MRCP liver elastography or serum fibrosis tests. B Do not use autoantibodies for risk stratification of patients with PSC. D As per BSG 2019 guidelines: Stratify patients based on noninvasive assessment. B Insufficient evidence to recommend a clinical score to predict individual patient prognosis. I 3. Diagnostic investigations Laboratory tests: As per EASL 2022 guidelines, consider obtaining serum IgG4 at the time of diagnosis in all adult patients with large-duct sclerosing cholangitis. C As per BSG 2019 guidelines: Obtain liver biochemical tests to identify cholestatic pattern in patients with suspected PSC. B Consider obtaining serum IgG4 levels to support the diagnosis of clinically suspected IgG4- related disease, but do not rely solely on elevated IgG4 levels for distinguishing IgG4- related sclerosing cholangitis from PSC. B As per ACG 2015 guidelines: Obtain testing for elevated serum IgG4 levels at least once in patients with PSC. B Consider obtaining antimitochondrial autoantibody testing to help exclude primary biliary cirrhosis in patients with PSC. C Diagnostic imaging: As per AASLD 2022 guidelines, obtain a 3D MRI with cholangiopancreatography with T1w and T2w axial images and contrast enhancement to evaluate for cholangiographic features https://web.pathway.md/diseases/recCAfARwQnJIjXTi 3/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway of PSC, including intrahepatic and/or extrahepatic strictures alternating with normal or slightly dilated segments, in patients with suspected PSC. E As per BSG 2019 guidelines: Obtain MRCP as the principal imaging modality for the evaluation of patients with suspected PSC. A Obtain noninvasive investigations such as MRCP, dynamic liver MRI and/or contrast CT in patients with new or changing symptoms or evolving abnormalities in laboratory investigations. B As per ACG 2015 guidelines, obtain MRCP, rather than ERCP, as the first-line investigation to establish the diagnosis of PSC. B Evaluation for osteoporosis: As per AASLD 2022 guidelines, obtain bone density testing to exclude osteopenia or osteoporosis at diagnosis and at 2-3-year intervals thereafter based on risk factors. E As per EASL 2022 guidelines, obtain DEXA at the time of diagnosis in all patients with PSC. Follow current evidence-based clinical practice guidelines for monitoring and treatment of osteopenia and osteoporosis. B As per BSG 2019 guidelines, obtain risk assessment for osteoporosis in all patients with PSC. Treat and follow-up in accordance with national guidelines once osteoporosis is detected. B As per ACG 2015 guidelines, obtain bone mineral density screening with DEXA at diagnosis in patients with PSC, and repeat at 2-4-year intervals. B Evaluation for autoimmune hepatitis: obtain further testing for AIH in < 25 years old patients with PSC, and in patients with disproportionate increases in aminotransferase levels (usually > 5 times the ULN). B Evaluation for cholangiocarcinoma: obtain contrast-enhanced, cross-sectional imaging as initial preferred investigation for the diagnosis and staging of patients with suspected cholangiocarcinoma. A Show 2 more Nutritional assessment: obtain nutritional assessments, including but not limited to biometrics and lipid-soluble vitamin levels, at PSC diagnosis and yearly thereafter with nutritional intervention and vitamin supplementation as needed. E Evaluation of fatigue: evaluate for and treat alternative causes of fatigue in patients with PSC. B Assessment of quality of life: assess the quality of life in patients with PSC as part of routine standard of care. B 4. Diagnostic procedures Liver biopsy: As per AASLD 2022 guidelines, consider performing liver biopsy to rule out small-duct PSC in patients with suspected PSC and a normal, high-quality MRI with cholangiopancreatography. E https://web.pathway.md/diseases/recCAfARwQnJIjXTi 4/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway Show 2 more As per EASL 2022 guidelines: Perform liver biopsy in adult patients with suspected PSC with normal high-quality MRCP, to confirm or exclude small-duct PSC. B Consider performing liver biopsy in patients with PSC and coexisting features of AIH, including markedly elevated transaminases, high IgG levels, and positive autoantibodies compatible with AIH. B As per BSG 2019 guidelines: Reserve liver biopsy for possible small-duct PSC, assessment of suspected possible overlap variants, or if the diagnosis is unclear. B Obtain pathological sampling of suspicious strictures in patients undergoing ERCP for dominant strictures. A As per ACG 2015 guidelines: Avoid performing routine liver biopsy to make a diagnosis in patients with a suspicion of PSC based on diagnostic cholangiographic findings. D Perform liver biopsy in patients with suspected small-duct PSC, or to exclude other conditions such as suspected overlap with AIH. B Endoscopic retrograde cholangiopancreatography: As per AASLD 2022 guidelines, avoid performing ERCP for the diagnosis of PSC. D Show 3 more As per BSG 2019 guidelines, consider performing ERCP in patients with biliary strictures requiring tissue acquisition (such as cytological brushings) or when therapeutic intervention is indicated. B Show 3 more As per ACG 2015 guidelines: Perform ERCP (with cytology, biopsies, and fluorescence in-situ hybridization) to exclude cholangiocarcinoma in patients with a dominant stricture on imaging. B Administer antibiotic prophylaxis in patients with PSC undergoing ERCP to prevent post- ERCP cholangitis. B Upper gastrointestinal endoscopy: As per AASLD 2022 guidelines, perform upper endoscopy to screen for varices if the liver stiffness is > 20 kPa by transient elastography or the platelet count is 150, 000/mm . E As per BSG 2019 guidelines, perform endoscopic evaluation for esophageal varices according to the international guidelines if there is evidence of cirrhosis and/or portal hypertension. A As per ACG 2015 guidelines, screen for esophageal varices in patients with signs of advanced disease with platelet counts < 150 10 /dL. B Lower gastrointestinal endoscopy: As per AASLD 2022 guidelines, perform ileocolonoscopy with biopsies in patients with newly diagnosed PSC and no previous diagnosis of IBD. Consider performing subsequent ileocolonoscopy at 5-year intervals or whenever symptoms suggestive of IBD occur in patients without IBD. E https://web.pathway.md/diseases/recCAfARwQnJIjXTi 5/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway As per EASL 2022 guidelines, perform ileocolonoscopy with biopsies from all colonic segments including the terminal ileum, regardless of the presence of lesions, at the time of PSC diagnosis. B As per BSG 2019 guidelines, perform colonoscopy and colonic biopsy for the evaluation of colitis in all patients with PSC. B As per ACG 2015 guidelines, perform a full colonoscopy with biopsies at the time of diagnosis in patients with PSC, regardless of the presence of symptoms, to assess for associated colitis. B 5. Medical management General principles: As per AASLD 2022 guidelines, consider offering enrollment in clinical trials in all patients with PSC. E As per BSG 2019 guidelines: Consider providing care involving 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. C Consider managing patients with early stable PSC in general clinics. B As per ACG 2015 guidelines, insufficient evidence to recommend a medical treatment for patients with PSC. I Management of pruritus: As per AASLD 2022 guidelines: Initiate bile acid sequestrants as initial therapy in patients with PSC presenting with pruritus and not responding to conservative measures, such as heat avoidance, emollients, and antihistamines. E Offer the following as alternatives for refractory pruritus: sertraline 100 mg daily naltrexone titrated to a dose of 50-100 mg daily rifampin 150-300 mg BID. E As per EASL 2022 guidelines: Offer bezafibrate or rifampicin for the treatment of moderate-to-severe pruritus in patients with sclerosing cholangitis. B Exclude relevant bile duct strictures as the cause of progressive pruritus in patients with large-duct sclerosing cholangitis. Treat relevant strictures by endoscopic balloon dilatation or stenting (if balloon dilatation alone is insufficient) after brushing. B As per BSG 2019 guidelines: Consider offering cholestyramine (or similar medication) as first-line therapy for the management of pruritus in patients with PSC. C Consider offering rifampicin and naltrexone as second-line therapy options for the management of pruritus in patients with PSC. C https://web.pathway.md/diseases/recCAfARwQnJIjXTi 6/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway As per ACG 2015 guidelines, administer local skin treatment with emollients and/or antihistamines to reduce symptoms in patients with PSC and mild pruritus. B Show 2 more Ursodeoxycholic acid: As per AASLD 2022 guidelines, consider initiating UDCA 13-23 mg/kg/day in patients not eligible or interested in clinical trials with persistently elevated ALP or GGT and continued if there is a meaningful reduction or normalization in ALP (GGT in pediatric patients) and/or symptoms improve with 12 months of treatment. E As per EASL 2022 guidelines: Consider offering UDCA at doses of 15-20 mg/kg/day to improve serum liver tests and surrogate markers of prognosis. B Do not use UDCA at doses of 28-30 mg/kg/day. D As per BSG 2019 guidelines, do not use routine UDCA for the treatment of patients with newly diagnosed PSC. D Show 2 more As per ACG 2015 guidelines, avoid using UDCA in doses > 28 mg/kg/day for the management of patients with PSC. D Corticosteroids: As per EASL 2022 guidelines, avoid using corticosteroids in the routine management of patients with PSC. D Show 2 more As per BSG 2019 guidelines: Do not use corticosteroids for the treatment of patients with classic PSC. D Consider initiating corticosteroids in patients with additional features of AIH or IgG4-related sclerosing cholangitis. B Immunosuppressants: As per EASL 2022 guidelines, avoid using immunosuppressives/biologics in the routine management of patients with PSC. D Show 2 more As per BSG 2019 guidelines, do not use immunosuppressants for the treatment of patients with classic PSC. D Vancomycin: insufficient evidence to support the use of oral vancomycin for the treatment of PSC. I 6. Nonpharmacologic interventions Vitamin supplements: consider setting a low threshold for empirical vitamin replacement in patients with advanced PSC, as poor nutrition and fat-soluble vitamin deficiencies are relatively common in these patients. C Psychosocial support: As per EASL 2022 guidelines, provide information on existing patient support groups. B https://web.pathway.md/diseases/recCAfARwQnJIjXTi 7/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway As per BSG 2019 guidelines, encourage patients with PSC to participate in patient support groups. B 7. Therapeutic procedures Endoscopic biliary dilatation: As per AASLD 2022 guidelines, decide on the choice between biliary balloon dilation with or without stenting at the discretion of the endoscopist. Remove stent within 4 weeks following placement if a plastic biliary stent is placed. E As per EASL 2022 guidelines, discuss the indication for endoscopic intervention ideally in multidisciplinary meetings of hepatologists, biliary endoscopists and abdominal radiologists, and performed by an experienced endoscopist if indicated. B Show 2 more As per BSG 2019 guidelines, perform biliary dilatation rather than insertion of biliary stents in patients undergoing ERCP for dominant strictures. B As per ACG 2015 guidelines: Perform ERCP with balloon dilatation in patients with PSC with dominant stricture and pruritus, and/or cholangitis, to relieve symptoms. B Avoid routine stenting after dilation of a dominant stricture, whereas consider short-term stenting in patients with severe stricture. D As per ASGE 2015 guidelines, perform ERCP with dilation and stent placement in patients with benign biliary strictures. B 8. Surgical interventions Liver transplantation: As per AASLD 2022 guidelines, consider offering liver transplantation in all patients with PSC and complications of end-stage liver disease, recurrent cholangitis, intractable pruritus, or early-stage hepatobiliary cancers. E As per EASL 2022 guidelines: Consider performing liver transplantation in patients with PSC and any of the following: decompensated cirrhosis or HCC, according to standard guidelines B recurrent bacterial cholangitis and/or severe pruritus or jaundice despite endoscopic and pharmacological therapy B high-grade biliary dysplasia confirmed by cytology or ductal histology C early-stage cholangiocarcinoma, within the context of clinical trials. C Perform liver transplantation in PSC using a duct-to-duct anastomosis unless anatomical disease location or technical surgical factors warrant a Roux-en-Y hepaticojejunostomy. B As per BSG 2019 guidelines, assess eligibility and refer patients with PSC for liver transplantation according to the national guidelines. A As per ACG 2015 guidelines: https://web.pathway.md/diseases/recCAfARwQnJIjXTi 8/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway Perform liver transplantation, when possible, over medical therapy or surgical drainage in patients with PSC with decompensated cirrhosis, to prolong survival. B Refer patients for liver transplantation if the Model for End-Stage Liver Disease score is > 14. B 9. Specific circumstances Pediatric patients: use the same diagnostic workup in pediatric patients as in adult practice, based on MRCP as the diagnostic modality of choice. B Show 5 more Pregnant patients: recognize that pregnancy is not contraindicated in patients with PSC and the risk of unfavorable course is correlated with the degree of portal hypertension. Consider obtaining close fetal monitoring because of the increased risk of preterm birth and fetal demise associated with high fetal bile acid levels. B Show 2 more Patients with ABCB4 deficiency: consider initiating low-to-medium-dose UDCA (10-15 mg/kg/day) in patients with ABCB4 deficiency. C Patients with gallbladder polyps: As per AASLD 2022 guidelines, consider performing cholecystectomy in patients with advanced PSC with gallbladder polyps > 8 mm, preferably at an experienced center. Consider monitoring polyps 8 mm with ultrasound every 6 months. E As per EASL 2022 guidelines, perform cholecystectomy in patients with PSC with gallbladder polyps 8 mm in size and smaller polyps growing in size due to the high risk of malignancy or dysplasia. B As per BSG 2019 guidelines, treat gallbladder polyps, if identified on annual ultrasound, directed by specialist hepatopancreaticobiliary multidisciplinary meeting. B As per ACG 2015 guidelines, perform cholecystectomy in patients with PSC and gallbladder polyps > 8 mm to prevent the development of gallbladder adenocarcinoma. B Patients with inflammatory bowel disease: As per AASLD 2022 guidelines: Perform high-definition surveillance colonoscopy with biopsies starting at age of 15 years and repeat at 1-2-year intervals to evaluate for colonic dysplasia in patients with PSC and IBD. E Manage IBD in patients with PSC as in patients without PSC. Continue active management of IBD and surveillance of colon cancer in the post-transplant period. E As per EASL 2022 guidelines, treat PSC-related IBD in line with current practice guidelines with the goal of achieving mucosal healing. B Show 2 more Patients with acute bacterial cholangitis: As per EASL 2022 guidelines: Administer antibiotics in patients with acute bacterial cholangitis and perform subsequent biliary decompression if an underlying relevant stricture is present. B https://web.pathway.md/diseases/recCAfARwQnJIjXTi 9/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway Do not use long-term antibiotics for the treatment of patients with PSC in the absence of recurrent bacterial cholangitis. D Patients with bacterial cholangitis: As per AASLD 2022 guidelines: Administer antibiotics for the treatment of bacterial cholangitis and consider obtaining MRCP to rule out relevant strictures. E Perform ERCP for bacterial cholangitis if there is an inadequate response to antibiotics. E Patients with autoimmune hepatitis: treat patients with PSC with a concurrent diagnosis of AIH according to pertinent clinical guidelines. E Critically ill patients with secondary sclerosing cholangitis: consider performing endoscopic removal of biliary casts and initiating low-to-medium-dose UDCA (10-15 mg/kg/day) in critically ill patients with sclerosing cholangitis. C 10. Patient education Preconception counseling: As per EASL 2022 guidelines, consider providing preconception counseling in female patients with PSC wishing to become pregnant. C Show 2 more As per BSG 2019 guidelines, provide preconception counseling and specialist monitoring regarding a higher risk of maternal and fetal complications in patients with cirrhosis. B 11. Follow-up and surveillance Indications for referral: As per AASLD 2022 guidelines, refer patients with an equivocal MRI with cholangiopancreatography to an experienced center for consideration of a repeat high- quality MRI with cholangiopancreatography or liver biopsy. E As per EASL 2022 guidelines, provide initial expert consultation in patients with PSC, and refer patients with symptomatic and/or progressive PSC to an experienced center with ready access to PSC clinical trials and a dedicated multidisciplinary team. B As per BSG 2019 guidelines, refer patients with symptomatic, evolving or complex disease for expert multidisciplinary assessment. B Show 2 more Follow-up: As per AASLD 2022 guidelines, obtain histological and cholangiographic assessments to distinguish recurrent PSC from allograft rejection and/or biliary complications in patients with elevated liver enzymes after transplant. E Show 2 more As per EASL 2022 guidelines: Consider obtaining routine noninvasive liver surveillance, based on: https://web.pathway.md/diseases/recCAfARwQnJIjXTi 10/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway clinical review and standard serum liver tests, including bilirubin, albumin, ALP, AST, platelets, and PT every 6 or 12 months depending on risk stratification B liver elastography and/or serum fibrosis tests at least every 2-3 years B liver ultrasound and/or abdominal MRI/MRCP every year. C Consider diagnosing recurrent PSC based on progressive biliary strictures on cholangiography and/or histological findings compatible with PSC > 90 days after liver transplantation upon exclusion of other identifiable causes. C As per BSG 2019 guidelines, ensure lifelong follow-up in patients with PSC given the unpredictable disease course and the serious nature of the complications. B As per ACG 2015 guidelines, screen and monitor patients with advanced liver disease for fat- soluble vitamin deficiencies. B Surveillance for inflammatory bowel disease: As per AASLD 2022 guidelines, consider performing subsequent ileocolonoscopy at 5-year intervals or whenever symptoms suggestive of IBD occur in patients without IBD. E As per EASL 2022 guidelines, consider performing a diagnostic colonoscopy every 5 years in patients with PSC without IBD or whenever complaints suspicious of IBD occur. C Surveillance for gallbladder polyps: consider obtaining annual ultrasound of the gallbladder in patients with PSC. Treat gallbladder polyps, if identified, directed by specialist hepatopancreaticobiliary multidisciplinary meeting. C Surveillance for malignancy, cholangiocarcinoma: As per ILCA/EASL 2023 guidelines, consider obtaining annual surveillance for cholangiocarcinoma with noninvasive radiologic modalities, preferably with MRI plus MRCP, in patients with PSC. C As per AASLD 2022 guidelines: Obtain annual surveillance for cholangiocarcinoma with abdominal imaging, preferably MRI with cholangiopancreatography, with or without serum CA19-9. E Do not obtain surveillance in patients with PSC < 18 years of age or with small-duct PSC. D As per EASL 2022 guidelines, consider obtaining surveillance for cholangiocarcinoma and gallbladder malignancy with ultrasound and/or MRI/MRCP at least annually in patients with large-duct disease, regardless of disease stage. Avoid obtaining CA 19-9 for surveillance purposes due to its insufficient accuracy. C As per AGA 2019 guidelines, consider obtaining surveillance for cholangiocarcinoma (with ultrasound, CT, or MRI, with or without serum CA19-9) every 6-12 months in all adult patients with PSC regardless of disease stage, especially in the first year after diagnosis, in patients with ulcerative colitis, and in patients diagnosed at an older age. C Show 4 more As per BSG 2019 guidelines, do not obtain routine measurement of serum CA 19-9 for surveillance for cholangiocarcinoma in patients with PSC. D As per ACG 2015 guidelines, consider screening patients with PSC for cholangiocarcinoma with regular cross-sectional imaging (ultrasound or MRI) and serial CA 19-9 every 6-12 months. C https://web.pathway.md/diseases/recCAfARwQnJIjXTi 11/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway Surveillance for malignancy, hepatocellular carcinoma: As per AASLD 2022 guidelines, obtain surveillance for HCC in patients with PSC with cirrhosis according to pertinent clinical guidelines. E As per EASL 2022 guidelines, consider obtaining surveillance for hepatobiliary malignancy every 6 months in the presence of cirrhosis. C As per AGA 2019 guidelines, obtain surveillance for HCC with ultrasound, CT, or MRI, with or without AFP, every 6 months in patients with PSC with cirrhosis. B As per BSG 2019 guidelines, obtain surveillance for HCC in patients with PSC, in the presence of cirrhosis, in accordance with international guidelines. B Surveillance for malignancy, gallbladder carcinoma: As per AASLD 2022 guidelines: Obtain annual surveillance for gallbladder carcinoma with abdominal imaging, preferably MRI with cholangiopancreatography, with or without serum CA19-9. E Do not obtain surveillance in patients with PSC < 18 years of age or with small-duct PSC. D As per AGA 2019 guidelines: Consider obtaining surveillance for gallbladder cancer (with ultrasound, CT, or MRI, with or without serum CA19-9) every 6-12 months in all adult patients with PSC regardless of disease stage, especially in the first year after diagnosis, in patients with ulcerative colitis, and in patients diagnosed at an older age. C Decide on performing cholecystectomy in patients with a gallbladder polyp based on the size and growth of the polyp, as well as the clinical status of the patient, with the knowledge of the increased risk of gallbladder cancer in polyps > 8 mm. B Surveillance for malignancy, colorectal cancer: As per EASL 2022 guidelines, perform annual surveillance (or every 1-2 years in individual patients without inflammatory activity) colonoscopy with biopsies in all adult patients with PSC-IBD regardless of the duration of IBD or liver transplant status. B As per BSG 2019 guidelines: Perform annual surveillance colonoscopy from the time of diagnosis of colitis in patients with PSC and coexistent IBD, according to the British Society of Gastroenterology guidelines. A Consider performing surveillance colonoscopy every 5 years or earlier in the advent of new symptoms in patients with PSC without IBD. C As per ACG 2015 guidelines, obtain annual surveillance for colorectal cancer in patients with PSC and colitis, beginning at the time of PSC diagnosis. B Clinical findings Symptoms Past medical history Chills Crohn's disease Fatigue IBD https://web.pathway.md/diseases/recCAfARwQnJIjXTi 12/14 6/29/23, 11:30 PM Primary sclerosing cholangitis Pathway Fever Ulcerative colitis Generalized pruritus Integument exam Infection RUQ pain Jaundice Steatorrhea Weight loss Family history PBC PSC Lab findings serum ALP serum ALT serum AST serum IgG serum IgM serum gamma- References 1. Lindor KD, Kowdley KV, Harrison ME. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol. 2015 May;110 5 646 59. Open 2. European Association for the Study of the Liver. EASL Clinical Practice Guidelines on Sclerosing Cholangitis. J Hepatol. 2022 May 31;S0168 8278 22 00326 9. Open 3. Christopher L Bowlus, Lionel Arriv , Annika Bergquist et al. AASLD Practice Guidance on Primary Sclerosing Cholangitis and Cholangiocarcinoma. Hepatology. 2022 Sep 9. Open 4. Chapman MH, Thorburn D, Hirschfield GM et al. British Society of Gastroenterology and UK PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut. 2019 Aug;68 8 1356 1378. Open 5. European Association for the Study of the Liver. EASL ILCA Clinical Practice Guidelines on Intrahepatic Cholangiocarcinoma. J Hepatol. 2023 Mar 22;S0168 8278 23 00185 X. Open 6. ASGE Standards of Practice Committee, Krishnavel V Chathadi, Vinay Chandrasekhara et al. The role of ERCP in benign diseases of the biliary tract. 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Monika Sarkar, Carla W Brady, Jaquelyn Fleckenstein et al. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 318 365. Open 17. Jean-Marc Dumonceau, Andrea Tringali, Ioannis S Papanikolaou et al. Endoscopic biliary stenting: indications, choice of stents, and results: European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline Updated October 2017. Endoscopy. 2018 Sep;50 9 910 930. Open https://web.pathway.md/diseases/recCAfARwQnJIjXTi 14/14