PSC-IBD: specific phenotype of inflammatory bowel disease associated with primary sclerosing cholangitis

Česká verzia

Authors: Lukáš Bajer; Pavel Wohl; Pavel Drastich
Authors place of work: Klinika hepatogastroenterologie IKEM, Praha
Published in the journal: Vnitř Lék 2018; 64(6): 659-664
Category: Reviews

Summary

Primary sclerosing cholangitis (PSC) is a progressive disease of the biliary tree which represents one of the most frequent indications for orthotopic liver transplantation (OLTx) in developed countries. LTx is currently the only curative procedure for PSC. Concomitant inflammatory bowel disease (IBD) is present in approximately 70 % of patients with PSC. Such colitis in PSC has specific clinical and etiological features and is considered as a distinct phenotype of IBD often referred to as “PSC-IBD”. The phenotype usually manifests as mild extensive colitis with right-sided predominance. Other morphological features, such as back- wash ileitis and rectal sparing, are also increasingly prevalent in PSC-IBD. Moreover, patients with PSC-IBD have increased risk towards developing colorectal neoplasia which tend to be highly progressive. Therefore, total colonoscopy employing multiple random biopsies or chromoendoscopy should be performed every 1–2 years once PSC-IBD is diagnosed. Even detection of low-grade dysplasia in colonic mucosa should lead towards prophylactic colectomy consideration in patients with PSC-IBD. Recommendations regarding pharmacological therapy are based on the similar principles as in Crohn´s disease and ulcerative colitis. However, status of the liver disease should always be taken into account. Proctocolectomy with ileal pouch-anal anastomosis (IPAA) is a method of choice in the field of surgical therapy of PSC-IBD. The clinical course of PSC-IBD may deteriorate after OLTx despite immunosuppressive therapy administration. IBD can even develop de-novo after OLTx and may significantly influence the risk ratio for PSC recurrence in the liver graft.

Key words:

inflammatory bowel disease – primary sclerosing cholangitis – PSC-IBD

Zdroje

Schrumpf E, Abdelnoor M, Fausa O et al. Risk factors in primary sclerosing cholangitis. J Hepatol 1994; 21(6): 1061–1066.
Escorsell A, Pares A, Rodes J et al. Epidemiology of primary sclerosing cholangitis in Spain. Spanish Association for the Study of the Liver. J Hepatol 1994; 21(5): 787–791.
Molodecky NA, Kareemi H, Parab R et al. Incidence of primary sclerosing cholangitis: a systematic review and meta-analysis. Hepatology 2011; 53(5): 1590–1599. Dostupné z DOI: <http://dx.doi.org/10.1002/hep.24247>.
Alipour M, Zaidi D, Valcheva R et al. Mucosal Barrier Depletion and Loss of Bacterial Diversity are Primary Abnormalities in Paediatric Ulcerative Colitis. J Crohns Colitis 2016; 10(4): 462–471. Dostupné z DOI: <http://dx.doi.org/10.1093/ecco-jcc/jjv223>.
Warren KW, Athanassiades S, Monge JI. Primary sclerosing cholangitis. A study of forty-two cases. Am J Surg 1966; 111(1): 23–38.
Rojas-Feria M, Castro M, Suarez E et al. Hepatobiliary manifestations in inflammatory bowel disease: the gut, the drugs and the liver. World J Gastroenterol 2013; 19(42): 7327–7340. Dostupné z DOI: <http://dx.doi.org/10.3748/wjg.v19.i42.7327>.
Schaeffer DF, Win LL, Hafezi-Bakhtiari S et al. The phenotypic expression of inflammatory bowel disease in patients with primary sclerosing cholangitis differs in the distribution of colitis. Dig Dis Sci 2013; 58(9): 2608–2614. Dostupné z DOI: <http://dx.doi.org/10.1007/s10620–013–2697–7>.
Sinakos E, Samuel S, Enders F et al. Inflammatory bowel disease in primary sclerosing cholangitis: a robust yet changing relationship. Inflamm Bowel Dis 2013; 19(5): 1004–1009. Dostupné z DOI: <http://dx.doi.org/10.1097/MIB.0b013e3182802893>.
Bajer L, Kverka M, Kostovcik M et al. Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis. World J Gastroenterol 2017; 23(25): 4548–4558. <http://dx.doi.org/10.3748/wjg.v23.i25.4548>.
de Vries AB, Janse M, Blokzijl H et al. Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis. World J Gastroenterol 2015; 21(6): 1956–1971. Dostupné z DOI: <http://dx.doi.org/10.3748/wjg.v21.i6.1956>.
Loftus EV Jr, Harewood GC, Loftus CG et al. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005; 54(1): 91–96. Dostupné z DOI: <http://dx.doi.org/10.1136/gut.2004.046615>.
Williamson KD, Chapman RW. Primary sclerosing cholangitis. Dig Dis 2014; 32(4): 438–445. <http://dx.doi.org/10.1159/000358150>.
Naess S, Bjornsson E, Anmarkrud JA et al. Small duct primary sclerosing cholangitis without inflammatory bowel disease is genetically different from large duct disease. Liver Int 2014; 34(10): 1488–1495. Dostupné z DOI: <http://dx.doi.org/10.1111/liv.12492>.
Williamson KD, Chapman RW. Primary sclerosing cholangitis: a clinical update. Br Med Bull 2015; 114(1): 53–64. Dostupné z DOI: <http://dx.doi.org/10.1093/bmb/ldv019>.
Chapman R, Fevery J, Kalloo A et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010; 51(2): 660–678. Dostupné z DOI: <http://dx.doi.org/10.1002/hep.23294>.
Palmela C, Peerani F, Castaneda D et al. Inflammatory Bowel Disease and Primary Sclerosing Cholangitis: A Review of the Phenotype and Associated Specific Features. Gut Liver 2018; 12(1): 17–29. Dostupné z DOI: <http://dx.doi.org/10.5009/gnl16510>.
Magro F, Gionchetti P, Eliakim R et al. Third European Evidence-based Consensus on Diagnosis and Management of Ulcerative Colitis. Part 1: Definitions, Diagnosis, Extra-intestinal Manifestations, Pregnancy, Cancer Surveillance, Surgery, and Ileo-anal Pouch Disorders. J Crohns Colitis 2017; 11(6): 649–670. Dostupné z DOI: <http://dx.doi.org/10.1093/ecco-jcc/jjx008>.
Fevery J, Henckaerts L, Van Oirbeek R et al. Malignancies and mortality in 200 patients with primary sclerosering cholangitis: a long-term single-centre study. Liver Int 2012; 32(2): 214–222. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1478–3231.2011.02575.x>.
Claessen MM, Vleggaar FP, Tytgat KM et al. High lifetime risk of cancer in primary sclerosing cholangitis. J Hepatol 2009; 50(1): 158–164. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jhep.2008.08.013>.
Terg R, Sambuelli A, Coronel E et al. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis and the risk of developing malignancies. A large prospective study. Acta Gastroenterol Latinoam 2008; 38(1): 26–33.
Broome U, Lofberg R, Veress B et al. Primary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential. Hepatology 1995; 22(5): 1404–1408.
Ullman TA, Loftus EV Jr, Kakar S et al. The fate of low grade dysplasia in ulcerative colitis. Am J Gastroenterol 2002; 97(4): 922–927. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1572–0241.2002.05610.x>.
Connell WR, Lennard-Jones JE, Williams CB et al. Factors affecting the outcome of endoscopic surveillance for cancer in ulcerative colitis. Gastroenterology 1994; 107(4): 934–944.
Zisman TL, Rubin DT. Colorectal cancer and dysplasia in inflammatory bowel disease. World J Gastroenterol 2008; 14(17): 2662–2669.
Stolfi C, Pallone F, Monteleone G. Colorectal cancer chemoprevention by mesalazine and its derivatives. J Biomed Biotechnol 2012; 2012: 980458. Dostupné z DOI: <http://dx.doi.org/10.1155/2012/980458>.
Angulo P, Batts KP, Jorgensen RA et al. Oral budesonide in the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2000; 95(9): 2333–2337. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1572–0241.2000.02323.x>.
Pallavicino F, Pellicano R, Reggiani S et al. Inflammatory bowel diseases and primary sclerosing cholangitis: hepatic and pancreatic side effects due to azathioprine. Eur Rev Med Pharmacol Sci 2013; 17(1): 84–87.
Ghabril M, Bonkovsky HL, Kum C et al. Liver injury from tumor necrosis factor-alpha antagonists: analysis of thirty-four cases. Clin Gastroenterol Hepatol 2013; 11(5): 558–564. Dostupné z DOI: <http://dx.doi.org/10.1016/j.cgh.2012.12.025>.
Christensen B, Micic D, Gibson PR et al. Vedolizumab in patients with concurrent primary sclerosing cholangitis and inflammatory bowel disease does not improve liver biochemistry but is safe and effective for the bowel disease. Aliment Pharmacol Ther 2018; 47(6): 753–762. Dostupné z DOI: <http://dx.doi.org/10.1111/apt.14525>.
Van Assche G, Dignass A, Bokemeyer B et al. Second European evidence-based consensus on the diagnosis and management of ulcerative colitis part 3: special situations. J Crohns Colitis 2013; 7(1): 1–33. Dostupné z DOI: <http://dx.doi.org/10.1016/j.crohns.2012.09.005>.
[European Association for the Study of the Liver]. EASL Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009; 51(2): 237–267. <http://dx.doi.org/10.1016/j.jhep.2009.04.009>.
Lindor KD. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med 1997; 336(10): 691–695. Dostupné z DOI: <http://dx.doi.org/10.1056/NEJM199703063361003>.
Wang R, Leong RW. Primary sclerosing cholangitis as an independent risk factor for colorectal cancer in the context of inflammatory bowel disease: a review of the literature. World J Gastroenterol 2014; 20(27): 8783–8789. Dostupné z DOI: <http://dx.doi.org/10.3748/wjg.v20.i27.8783>.
Singh S, Khanna S, Pardi DS et al. 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): 1631–1638. Dostupné z DOI: <http://dx.doi.org/10.1097/MIB.0b013e318286fa61>.
Treeprasertsuk S, Bjornsson E, Sinakos E et al. Outcome of patients with primary sclerosing cholangitis and ulcerative colitis undergoing colectomy. World J Gastrointest Pharmacol Ther 2013; 4(3): 61–68. Dostupné z DOI: <http://dx.doi.org/10.4292/wjgpt.v4.i3.61>.
Wiesner RH, LaRusso NF, Dozois RR et al. Peristomal varices after proctocolectomy in patients with primary sclerosing cholangitis. Gastroenterology 1986; 90(2): 316–322.
Mathis KL, Benavente-Chenhalls LA, Dozois EJ et al. 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): 787–792. Dostupné z DOI: <http://dx.doi.org/10.1007/DCR.0b013e318217eea7>.
Block M, Jorgensen KK, Oresland T et al. Colectomy for patients with ulcerative colitis and primary sclerosing cholangitis – what next? J Crohns Colitis 2014; 8(5): 421–430. Dostupné z DOI: <http://dx.doi.org/10.1016/j.crohns.2013.10.008>.
Gorgun E, Remzi FH, Manilich E et al. Surgical outcome in patients with primary sclerosing cholangitis undergoing ileal pouch-anal anastomosis: a case-control study. Surgery 2005; 138(4): 631–637; discussion 637–639. <http://dx.doi.org/10.1016/j.surg.2005.07.014>.
Hirschfield GM, Karlsen TH, Lindor KD et al. Primary sclerosing cholangitis. Lancet 2013; 382(9904): 1587–1599. Dostupné z DOI: <http://dx.doi.org/10.1016/S0140–6736(13)60096–3>.
Marelli L, Xirouchakis E, Kalambokis G et al. Does the severity of primary sclerosing cholangitis influence the clinical course of associated ulcerative colitis? Gut 2011; 60(9): 1224–1228. Dostupné z DOI: <http://dx.doi.org/10.1136/gut.2010.235408>.
Moncrief KJ, Savu A, Ma MM et al. The natural history of inflammatory bowel disease and primary sclerosing cholangitis after liver transplantation – a single-centre experience. Can J Gastroenterol 2010; 24(1): 40–46.
Hildebrand T, Pannicke N, Dechene A et al. Biliary strictures and recurrence after liver transplantation for primary sclerosing cholangitis: A retrospective multicenter analysis. Liver Transpl 2016; 22(1): 42–52. Dostupné z DOI: <http://dx.doi.org/10.1002/lt.24350>.
Lindstrom L, Jorgensen KK, Boberg KM et al. Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation: a Nordic Multicentre Study. Scand J Gastroenterol 2018; 53(3): 297–304. Dostupné z DOI: <http://dx.doi.org/10.1080/00365521.2017.1421705>.