A clinical review and standard serum liver tests are recommended every 6-12 months, depending on risk stratification. The tests include bilirubin, albumin, alkaline phosphatase, aspartate aminotransferase, platelet count, and prothrombin time. Every 2-3 years, liver elastography and/or serum fibrosis tests are recommended. Every year, liver ultrasound and/or abdominal magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) are suggested.[2]European Association for the Study of the Liver. EASL clinical practice guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806.
https://www.journal-of-hepatology.eu/article/S0168-8278(22)00326-9/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/35738507?tool=bestpractice.com
Bone mineral density scanning using dual energy x-ray absorptiometry is suggested in all patients at diagnosis and at 2- to 3- or 4-year intervals (based on risk factors) to exclude osteoporosis.[2]European Association for the Study of the Liver. EASL clinical practice guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806.
https://www.journal-of-hepatology.eu/article/S0168-8278(22)00326-9/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/35738507?tool=bestpractice.com
[3]Bowlus CL, Arrivé L, Bergquist A, et al. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology. 2023 Feb 1;77(2):659-702.
https://journals.lww.com/hep/fulltext/2023/02000/aasld_practice_guidance_on_primary_sclerosing.29.aspx
[29]Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010 Feb;51(2):660-78.
https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.23294
http://www.ncbi.nlm.nih.gov/pubmed/20101749?tool=bestpractice.com
[30]Lindor KD, Kowdley KV, Harrison ME; American College of Gastroenterology. ACG clinical guideline: primary sclerosing cholangitis. Am J Gastroenterol. 2015 May;110(5):646-59.
https://gi.org/guideline/primary-sclerosing-cholangitis
http://www.ncbi.nlm.nih.gov/pubmed/25869391?tool=bestpractice.com
[37]Zein CO, Jorgensen RA, Clarke B, et al. Alendronate improves bone mineral density in primary biliary cirrhosis: a randomized placebo-controlled trial. Hepatology. 2005 Oct;42(4):762-71.
https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.20866
http://www.ncbi.nlm.nih.gov/pubmed/16175618?tool=bestpractice.com
[38]Collier J. Bone disorders in chronic liver disease. Hepatology. 2007 Oct;46(4):1271-8.
https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.21852
http://www.ncbi.nlm.nih.gov/pubmed/17886334?tool=bestpractice.com
Periodic laboratory testing to evaluate for fat-soluble vitamin deficiencies is recommended.[29]Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010 Feb;51(2):660-78.
https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.23294
http://www.ncbi.nlm.nih.gov/pubmed/20101749?tool=bestpractice.com
Screening for hepatocellular carcinoma in patients with cirrhosis typically includes liver ultrasound and serum alpha-fetoprotein measurement every 6 months.[104]Bowlus CL, Lim JK, Lindor KD. AGA clinical practice update on surveillance for hepatobiliary cancers in patients with primary sclerosing cholangitis: expert review. Clin Gastroenterol Hepatol. 2019 Nov;17(12):2416-22.
https://www.cghjournal.org/article/S1542-3565(19)30744-X/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/31306801?tool=bestpractice.com
[105]Suddle A, Reeves H, Hubner R, et al. British Society of Gastroenterology guidelines for the management of hepatocellular carcinoma in adults. Gut. 2024 Jul 11;73(8):1235-68.
https://gut.bmj.com/content/73/8/1235.long
http://www.ncbi.nlm.nih.gov/pubmed/38627031?tool=bestpractice.com
Screen for serum antibodies to hepatitis A and hepatitis B virus so that immunisation can be offered if there is no evidence of previous infection or immunisation.
In patients with primary sclerosing cholangitis who do not have, or are not diagnosed with, inflammatory bowel disease (IBD), ileocolonoscopy should be repeated (US guidance) or considered (European guidelines) every 5 years, or whenever they have symptoms suggestive of IBD.[2]European Association for the Study of the Liver. EASL clinical practice guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806.
https://www.journal-of-hepatology.eu/article/S0168-8278(22)00326-9/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/35738507?tool=bestpractice.com
[3]Bowlus CL, Arrivé L, Bergquist A, et al. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology. 2023 Feb 1;77(2):659-702.
https://journals.lww.com/hep/fulltext/2023/02000/aasld_practice_guidance_on_primary_sclerosing.29.aspx
From the age of 15 years, all patients with co-existing IBD should undergo high-definition surveillance colonoscopy regularly due to a high risk of colorectal cancer. US guidance suggests 1- to 2-year surveillance intervals, whereas European guidelines recommend surveillance annually, with 1- to 2-year intervals if there is no inflammatory activity.[2]European Association for the Study of the Liver. EASL clinical practice guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806.
https://www.journal-of-hepatology.eu/article/S0168-8278(22)00326-9/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/35738507?tool=bestpractice.com
[3]Bowlus CL, Arrivé L, Bergquist A, et al. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology. 2023 Feb 1;77(2):659-702.
https://journals.lww.com/hep/fulltext/2023/02000/aasld_practice_guidance_on_primary_sclerosing.29.aspx
[29]Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010 Feb;51(2):660-78.
https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.23294
http://www.ncbi.nlm.nih.gov/pubmed/20101749?tool=bestpractice.com
[30]Lindor KD, Kowdley KV, Harrison ME; American College of Gastroenterology. ACG clinical guideline: primary sclerosing cholangitis. Am J Gastroenterol. 2015 May;110(5):646-59.
https://gi.org/guideline/primary-sclerosing-cholangitis
http://www.ncbi.nlm.nih.gov/pubmed/25869391?tool=bestpractice.com
[39]Vleggaar FP, Lutgens MW, Claessen MM, et al. Review article: the relevance of surveillance endoscopy in long-lasting inflammatory bowel disease. Aliment Pharmacol Ther. 2007 Dec;26 Suppl 2:47-52.
http://www.ncbi.nlm.nih.gov/pubmed/18081648?tool=bestpractice.com
Surveillance endoscopy after liver transplantation can continue as per the pre-transplant recommendations.[2]European Association for the Study of the Liver. EASL clinical practice guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806.
https://www.journal-of-hepatology.eu/article/S0168-8278(22)00326-9/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/35738507?tool=bestpractice.com
[3]Bowlus CL, Arrivé L, Bergquist A, et al. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology. 2023 Feb 1;77(2):659-702.
https://journals.lww.com/hep/fulltext/2023/02000/aasld_practice_guidance_on_primary_sclerosing.29.aspx