Evidence-based

References

Every statement on ibdpsc.org is grounded in the published medical literature. Below, the key points are grouped by topic and linked to the peer-reviewed studies that support them. Sources were retrieved from a curated IBD with primary sclerosing cholangitis research library and verified against the U.S. National Library of Medicine (PubMed); none are retracted. Last verified July 2026.

How common

Roughly two-thirds of people with PSC have IBD, and PSC develops in a small percentage of people with ulcerative colitis.9, 10

PSC-IBD: the distinct colitis

The colitis of IBD-PSC is a distinct phenotype, often extensive yet mild and most active on the right side. Rectal sparing and backwash ileitis are common in some studies and uncommon in others.1–3, 11, 12

Colorectal cancer: risk & surveillance

IBD-PSC carries a markedly higher colorectal-cancer risk, more often right-sided, which is why annual surveillance colonoscopy begins at PSC diagnosis.4–6, 14, 15

Biliary & gallbladder cancer

PSC also raises the risk of bile-duct (cholangiocarcinoma) and gallbladder cancer, monitored separately by the liver team.16–18

Diagnosis & natural history of PSC

PSC is a chronic, slowly progressive bile-duct disease strongly linked to inflammatory bowel disease.7, 8

Liver disease: management & transplant

PSC is followed by a hepatologist with liver tests and MRCP; for advanced disease, liver transplantation is the most effective treatment.13, 19–21, 24, 25

Colitis therapy in PSC-IBD

Day-to-day colitis in IBD-PSC is managed with the same medical therapies used for IBD, guided by disease activity.31, 32

Surgery: colectomy, IPAA & the pouch

People with IBD-PSC who need surgery can undergo ileal pouch-anal anastomosis; chronic pouchitis is more common in PSC and timing is coordinated with the liver disease.22, 23, 26–30

Children with PSC-IBD

PSC-IBD also occurs in children and is followed with the same attention to colon and liver.33, 34

References

  1. Boonstra K, van Erpecum KJ, van Nieuwkerk KM, Drenth JP, Poen AC, Witteman BJ, Tuynman HA, Beuers U, Ponsioen CY. Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease. Inflamm Bowel Dis. 2012;18(12):2270-6. PMID: 22407885.
  2. Ricciuto A, Kamath BM, Griffiths AM. The IBD and PSC Phenotypes of PSC-IBD. Curr Gastroenterol Rep. 2018;20(4):16. PMID: 29594739.
  3. de Vries AB, Janse M, Blokzijl H, Weersma RK. Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis. World J Gastroenterol. 2015;21(6):1956-71. PMID: 25684965.
  4. Zheng HH, Jiang XL. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease: a meta-analysis of 16 observational studies. Eur J Gastroenterol Hepatol. 2016;28(4):383-90. PMID: 26938805.
  5. Das TS, Ho K, Udaikumar J, Chen B, Delau O, Shaukat A, Jacobson I, Sarwar R. Risk of colorectal cancer in patients with primary sclerosing cholangitis and concomitant inflammatory bowel disease compared with primary sclerosing cholangitis only. Hepatol Res. 2024;54(9):807-816. PMID: 38419394.
  6. Claessen MM, Lutgens MW, van Buuren HR, Oldenburg B, Stokkers PC, van der Woude CJ, Hommes DW, de Jong DJ, Dijkstra G, van Bodegraven AA, Siersema PD, Vleggaar FP. More right-sided IBD-associated colorectal cancer in patients with primary sclerosing cholangitis. Inflamm Bowel Dis. 2009;15(9):1331-6. PMID: 19229982.
  7. Fricker ZP, Lichtenstein DR. Primary Sclerosing Cholangitis: A Concise Review of Diagnosis and Management. Dig Dis Sci. 2019;64(3):632-642. PMID: 30725292.
  8. Wiesner RH, Grambsch PM, Dickson ER, Ludwig J, MacCarty RL, Hunter EB, Fleming TR, Fisher LD, Beaver SJ, LaRusso NF. Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis. Hepatology. 1989;10(4):430-6. PMID: 2777204.
  9. Zhang Y, Gao X, He Z, Jia H, Chen M, Wang X, Hong L, Cui Y, Wan J. Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta-analysis. Liver Int. 2022;42(8):1814-1822. PMID: 35689520.
  10. Barberio B, Massimi D, Cazzagon N, Zingone F, Ford AC, Savarino EV. Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis. Gastroenterology. 2021;161(6):1865-1877. PMID: 34425093.
  11. Loftus EV Jr, Harewood GC, Loftus CG, Tremaine WJ, Harmsen WS, Zinsmeister AR, Jewell DA, Sandborn WJ. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut. 2005;54(1):91-6. PMID: 15591511.
  12. Murasugi S, Ito A, Omori T, Nakamura S, Tokushige K. Clinical Characterization of Ulcerative Colitis in Patients with Primary Sclerosing Cholangitis. Gastroenterol Res Pract. 2020;2020:7969628. PMID: 33224192.
  13. Eaton JE, Silveira MG, Pardi DS, Sinakos E, Kowdley KV, Luketic VA, Harrison ME, McCashland T, Befeler AS, Harnois D, Jorgensen R, Petz J, Lindor KD. High-dose ursodeoxycholic acid is associated with the development of colorectal neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Am J Gastroenterol. 2011;106(9):1638-45. PMID: 21556038.
  14. Sokol H, Chazouilleres O. Colorectal neoplasia in PSC-IBD patients: are times changing?. Liver Int. 2012;32(2):175-6. PMID: 22226230.
  15. Jamil OK, Shaw D, Deng Z, Dinardi N, Fillman N, Khanna S, Krugliak Cleveland N, Sakuraba A, Weber CR, Cohen RD, Dalal S, Jabri B, Rubin DT, Pekow J. Inflammation in the proximal colon is a risk factor for the development of colorectal neoplasia in inflammatory bowel disease patients with primary sclerosing cholangitis. Ther Adv Gastroenterol. 2023;16:17562848231184985. PMID: 37692199.
  16. Villard C, Friis-Liby I, Rorsman F, Said K, Warnqvist A, Cornillet M, Kechagias S, Nyhlin N, Werner M, Janczewska I, Hagström T, Nilsson E, Bergquist A. Prospective surveillance for cholangiocarcinoma in unselected individuals with primary sclerosing cholangitis. J Hepatol. 2023;78(3):604-613. PMID: 36410555.
  17. Horsley-Silva JL, Rodriguez EA, Franco DL, Lindor KD. An update on cancer risk and surveillance in primary sclerosing cholangitis. Liver Int. 2017;37(8):1103-1109. PMID: 28028930.
  18. Buckles DC, Lindor KD, Larusso NF, Petrovic LM, Gores GJ. In primary sclerosing cholangitis, gallbladder polyps are frequently malignant. Am J Gastroenterol. 2002;97(5):1138-42. PMID: 12014717.
  19. Leung KK, Deeb M, Fischer SE, Gulamhusein A. Recurrent Primary Sclerosing Cholangitis: Current Understanding, Management, and Future Directions. Semin Liver Dis. 2021;41(3):409-420. PMID: 34182588.
  20. Chapman RW, Williamson KD. Are Dominant Strictures in Primary Sclerosing Cholangitis a Risk Factor for Cholangiocarcinoma?. Curr Hepatol Rep. 2017;16(2):124-129. PMID: 28706774.
  21. Barkin JA, Levy C, Souto EO. Endoscopic Management of Primary Sclerosing Cholangitis. Ann Hepatol. 2017;16(6):842-850. PMID: 29055922.
  22. Mathis KL, Benavente-Chenhalls LA, Dozois EJ, Wolff BG, Larson DW. Short- and long-term surgical outcomes in patients undergoing proctocolectomy with ileal pouch-anal anastomosis in the setting of primary sclerosing cholangitis. Dis Colon Rectum. 2011;54(7):787-92. PMID: 21654244.
  23. Ong J, Bath MF, Swift C, Al-Naeeb Y. Does colectomy affect the progression of primary sclerosing cholangitis? A systematic review and meta-analysis. Gastroenterol Hepatol Bed Bench. 2018;11(4):277-283. PMID: 30425805.
  24. Singh S, Khanna S, Pardi DS, Loftus EV, Talwalkar JA. Effect of ursodeoxycholic acid use on the risk of colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease: a systematic review and meta-analysis. Inflamm Bowel Dis. 2013;19(8):1631-8. PMID: 23665966.
  25. Graziadei IW, Wiesner RH, Marotta PJ, Porayko MK, Hay JE, Charlton MR, Poterucha JJ, Rosen CB, Gores GJ, LaRusso NF, Krom RA. Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology. 1999;30(5):1121-7. PMID: 10534330.
  26. Wasmuth HH, Tranø G, Endreseth BH, Wibe A, Rydning A, Myrvold HE. Primary sclerosing cholangitis and extraintestinal manifestations in patients with ulcerative colitis and ileal pouch-anal anastomosis. J Gastrointest Surg. 2010;14(7):1099-104. PMID: 20480253.
  27. Imam MH, Eaton JE, Puckett JS, Loftus EV Jr, Mathis KL, Gossard AA, Talwalkar JA, Lindor KD. Neoplasia in the ileoanal pouch following colectomy in patients with ulcerative colitis and primary sclerosing cholangitis. J Crohns Colitis. 2014;8(10):1294-9. PMID: 24768559.
  28. Nordenvall C, Olén O, Johan Nilsson P, Ekbom A, Bottai M, Myrelid P, Bergquist A. Restorative Surgery in Patients With Primary Sclerosing Cholangitis and Ulcerative Colitis Following a Colectomy. Inflamm Bowel Dis. 2018;24(3):624-632. PMID: 29462381.
  29. Nordenvall C, Bergquist A. Editorial: the role of colonic inflammation in the progression of liver disease in primary sclerosing cholangitis-Authors' reply and Letter: the effects of colectomy prior to the diagnosis of primary sclerosing cholangitis on prognosis may have been overestimated. Aliment Pharmacol Ther. 2018;47(3):441-442. PMID: 29314129.
  30. Mol B, van Nieuwamerongen M, van Munster KN, Färkkilä M, Folseraas T, Tjønnfjord SKV, Hov JR, Boberg K, Vesterhus M, Jørgensen KK, Bergquist A, Goet JC, de Vries AC, van der Meer AJP, Weersma RK, Inderson A, Bogaards JA, Ponsioen CY, IPSCR study group. Proctocolectomy with permanent ileostomy is associated with improved transplant-free survival in patients with PSC. JHEP Rep. 2026;8(3):101700. PMID: 41757378.
  31. Shah A, Jones MP, Callaghan G, Fairlie T, Ma X, Culver EL, Stuart K, De Cruz P, O'Beirne J, Tabibian JH, Dignass A, Canbay A, Gores GJ, Holtmann GJ. Efficacy and safety of biologics in primary sclerosing cholangitis with inflammatory bowel disease: A systematic review and meta-analysis. Hepatol Commun. 2024;8(1). PMID: 38206197.
  32. Tse CS, Loftus EV, Raffals LE, Gossard AA, Lightner AL. Effects of vedolizumab, adalimumab and infliximab on biliary inflammation in individuals with primary sclerosing cholangitis and inflammatory bowel disease. Aliment Pharmacol Ther. 2018;48(2):190-195. PMID: 29808485.
  33. Miloh T, Arnon R, Shneider B, Suchy F, Kerkar N. A retrospective single-center review of primary sclerosing cholangitis in children. Clin Gastroenterol Hepatol. 2009;7(2):239-45. PMID: 19121649.
  34. Deneau MR, El-Matary W, Valentino PL, Abdou R, Alqoaer K, Amin M, Amir AZ, Auth M, Bazerbachi F, Broderick A, Chan A, Cotter J, Doan S, El-Youssef M, Ferrari F, Furuya KN, Gottrand M, Gottrand F, Gupta N, Homan M, Kamath BM, Kim KM, Kolho KL, Konidari A, Koot B, Iorio R, Ledder O, Mack C, Martinez M, Miloh T, Mohan P, O'Cathain N, Papadopoulou A, Ricciuto A, Saubermann L, Sathya P, Shteyer E, Smolka V, Tanaka A, Varier R, Venkat V, Vitola B, Vos MB, Woynarowski M, Yap J, Jensen MK. The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration. Hepatology. 2017;66(2):518-527. PMID: 28390159.

Compiled by IBDology. Citations are provided for transparency and education and are not a substitute for advice from your own care team.