Abstract
Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the biliary tree of unknown etiology leading to stricturing and dilation. There is currently no effective medical therapy for PSC and liver transplantation (LT) remains the ultimate treatment for severe disease defined as repeated episodes of cholangitis, decompensated biliary cirrhosis or in exceptional cases, cholangiocarcinoma (CCA). Patients often present with a “dominant” stricture and the therapeutic endoscopist plays an important role in management to improve biliary patency using a variety of techniques that involve sampling, balloon dilation and temporary stenting. Newer modalities such as self-expanding metal stents or magnetic compression anastomosis that have been used in other diseases may have a role to play in PSC but should remain investigational. Liver transplantation for PSC is curative in most cases but the optimal timing remains unclear. The lifetime risk of CCA is 10–15% in PSC patients and LT is often not possible at the time of diagnosis. Multiple studies have tried to identify risk factors and to diagnose CCA at an early stage when surgical resection may be possible or LT can be performed. However, deceased donor organs for LT remain in short supply throughout the world so even identifying PSC patients with CCA at an early stage may not be beneficial unless a live donor organ is available.
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References
Karlseon TH, Schrumpf E, Boberg KM. Primary sclerosing cholangitis. Best Pract Res Clin Gastroenterol 2010;24:655–666
Mendes F, Lindor KD. Primary sclerosing cholangitis: overview and update. Nat Rev Gastroenterol Hepatol 2010;7:611–619
Rizvi S, Eaton JE, Gores GJ. Primary sclerosing cholangitis as a premalignant biliary tract disease: surveillance and management. Clin Gastroenterol Hepatol 2015;13:2152–2165
Aabakken L, Karlsen TH, Albert J, Arvanitakis M, Chazouilleres O, Dumonceau JM, et al. Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline. Endoscopy 2017;49:588–608
Gluck M, Cantone NR, Brandabur JJ, et al. A twenty year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol 2008;42:1032–1039
Bjornsson E, Lindqvist-Ottosson J, Asztely M, et al. Dominant strictures in patients with primary sclerosing cholangitis. Am J Gastroenterol 2004;99:502–508
Alkhatib AA, Hilden K, Adler DG. Comorbidities, sphincterotomy, and balloon dilation predict post-ERCP adverse events in PSC patients: operator experience is protective. Dig Dis Sci 2011;56:3685–3688
Bangarulingam SY, Gossard AA, Petersen BT, et al. Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol 2009;104:855–860
Navaneethan U, Jegadeesan R, Nayak S, et al. ERCP-related adverse events in patients with primary sclerosing cholangitis. Gastrointest Endosc 2015;81:410–419
Ponsioen CY, Lam K, van Milligen de Wit AWM, et al. Four years experience with short term stenting in primary sclerosing cholangitis. Am J Gastroenterol 1999;94:2403–2407
Ponsioen CY, Arnelo U, Bergquist A, Rauws EA, Paulsen V, Cantú P, et al. No superiority of stents vs balloon dilatation for dominant strictures in patients with primary sclerosing cholangitis. Gastroenterology 2018. https://doi.org/10.1053/j.gastro.2018.05.034 (Epub ahead of print)
Almadi MA, Barkun A, Martel M. Plastic vs. self-expandable metal stents for palliation in malignant biliary obstruction: a series of meta-analyses. Am J Gastroenterol 2017;112:260–273
Conio M, Mangiavillano B, Caruso A, Filiberti RA, Baron TH, De Luca L, et al. Covered versus uncovered self-expandable metal stent for palliation of primary malignant extrahepatic biliary strictures: a randomized multicenter study. Gastrointest Endosc. 2018. https://doi.org/10.1016/j.gie.2018.03.029 (Epub ahead of print)
Hori Y, Naitoh I, Hayashi K, Ban T, Natsume M, Okumura F, et al. Predictors of stent dysfunction after self-expandable metal stent placement for malignant gastric outlet obstruction: tumor ingrowth in uncovered stents and migration of covered stents. Surg Endosc 2017;31:4165–4173
Khan MA, Baron TH, Kamal F, Ali B, Nollan R, Ismail MK, et al. Efficacy of self-expandable metal stents in management of benign biliary strictures and comparison with multiple plastic stents: a meta-analysis. Endoscopy 2017;49:682–694
Schmidt A, Pickartz T, Lerch MM, Fanelli F, Fiocca F, Lucatelli P, et al. Effective treatment of benign biliary strictures with a removable, fully covered, self-expandable metal stent: a prospective, multicenter European study. United European Gastroenterol J 2017;5:398–407
Saxena P, Diehl DL, Kumbhari V, Shieh F, Buscaglia JM, Sze W, et al. A US Multicenter study of safety and efficacy of fully covered self-expandable metallic stents in benign extrahepatic biliary strictures. Dig Dis Sci 2015;60:3442–3448
Park JK, Moon JH, Choi HJ, Min SK, Lee TH, Cheon GJ, et al. Anchoring of a fully covered self-expandable metal stent with a 5F double-pigtail plastic stent to prevent migration in the management of benign biliary strictures. Am J Gastroenterol 2011;106:1761–1765
Devière J, Nageshwar Reddy D, Püspök A, Ponchon T, Bruno MJ, Bourke MJ, et al. Successful management of benign biliary strictures with fully covered self-expanding metal stents. Gastroenterology 2014;147:385–395
Itoi T, Kasuya K, Sofuni A, Itokawa F, Tsuchiya T, Kurihara T, et al. Magnetic compression anastomosis for biliary obstruction: review and experience at Tokyo Medical University Hospital. J Hepatobiliary Pancreat Sci 2011;18:357–365
Takao S, Matsuo Y, Shinchi H, Nakajima S, Aikou T, Iseji T, et al. Magnetic compression anastomosis for benign obstruction of the common bile duct. Endoscopy 2001;33:988–990
Jang SI, Lee KH, Yoon HJ, Lee DK. Treatment of completely obstructed benign biliary strictures with magnetic compression anastomosis: follow-up results after recanalization. Gastrointest Endosc 2017;85:1057–1066
Parlak E, Koksal AS, Kucukay F, Eminler AT, Toka B, Uslan MI. A novel technique for the endoscopic treatment of complete biliary anastomosis obstructions after liver transplantation: through-the-scope magnetic compression anastomosis. Gastrointest Endosc 2017;85:841–847
Ersoz G, Tekin F, Bozkaya H, Parildar M, Turan I, Karasu Z, et al. Magnetic compression anastomosis for patients with a disconnected bile duct after living-donor related liver transplantation: a pilot study. Endoscopy 2016;48:652–656
Jang SI, Kim JH, Won JY, Lee KH, Kim HW, You JW, et al. Magnetic compression anastomosis is useful in biliary anastomotic strictures after living donor liver transplantation. Gastrointest Endosc 2011;74:1040–1048
Umemura A, Sasaki A, Nitta H, Takahara T, Hasegawa Y, Wakabayashi G. Magnetic compression anastomosis for the stricture of the choledochocholedochostomy after ABO-incompatible living donor liver transplantation. Clin J Gastroenterol 2014;7:361–364
Ponsioen CY, Chapman RW, Chazouillères O, Hirschfield GM, Karlsen TH, Lohse AW, et al. Surrogate endpoints for clinical trials in primary sclerosing cholangitis: Review and results from an International PSC Study Group consensus process. Hepatology 2016;63:1357–1367
Deneau MR, El-Matary W, Valentino PL, Abdou R, Alqoaer K, Amin M, et al. The natural history of primary sclerosing cholangitis in 781 children: a multicenter, international collaboration. Hepatology 2017;66:518–527
Weismüller TJ, Trivedi PJ, Bergquist A, Imam M, Lenzen H, Ponsioen CY, et al. Patient age, sex, and inflammatory bowel disease phenotype associate with course of primary sclerosing cholangitis. Gastroenterology 2017;152:1975–1984
Ji SG, Juran BD, Mucha S, Folseraas T, Jostins L, Melum E, et al. Genome-wide association study of primary sclerosing cholangitis identifies new risk loci and quantifies the genetic relationship with inflammatory bowel disease. Nat Genet 2017;49:269–273
Friedrich K, Smit M, Brune M, Giese T, Rupp C, Wannhoff A, et al. CD14 is associated with biliary stricture formation. Hepatology 2016;64:843–852
Vesterhus M, Holm A, Hov JR, Nygård S, Schrumpf E, Melum E, et al. Novel serum and bile protein markers predict primary sclerosing cholangitis disease severity and prognosis. J Hepatol 2017;66:1214–1222
de Vries EM, de Krijger M, Färkkilä M, Arola J, Schirmacher P, Gotthardt D, et al. Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: an international cohort study. Hepatology 2017;65:907–919
Vesterhus M, Hov JR, Holm A, Schrumpf E, Nygård S, Godang K, et al. Enhanced liver fibrosis score predicts transplant-free survival in primary sclerosing cholangitis. Hepatology 2015;62:188–197
Goldberg DS, Camp A, Martinez-Camacho A, Forman L, Fortune B, Reddy KR. Risk of waitlist mortality in patients with primary sclerosing cholangitis and bacterial cholangitis. Liver Transpl 2013;19:250–258
Martin EF, Levy C. Timing, Management, and outcomes of liver transplantation in primary sclerosing cholangitis. Semin Liver Dis 2017;37:305–313
Gordon FD, Goldberg DS, Goodrich NP, Lok AS, Verna EC, Selzner N, et al. Recurrent primary sclerosing cholangitis in the Adult-to-Adult Living Donor Liver Transplantation Cohort Study: Comparison of risk factors between living and deceased donor recipients. Liver Transpl 2016;22:1214–1222
Bergquist A, Ekbom A, Olsson R, et al. Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 2002;36:321–327
Boberg KM, Jebsen P, Clausen OP et al. diagnostic benefit of biliary brush cytology in cholangiocarcinoma in primary sclerosing cholangitis. J Hepatol 2006;45:568–574
Siqueira E, Schoen RE, Silverman W, et al. Detecting cholangiocarcinoma in patients with primary sclerosing cholangitis. Gastrointest Endosc. 2002;56:40–47
Lindberg B, Arnelo U, Bergquist A, et al. Diagnosis of biliary strictures in conjunction with endoscopic retrograde cholangiopancreaticography, with special reference to patients with primary sclerosing cholangitis. Endoscopy 2002;34:909–916
Eaton JE, Barr Fritcher EG, Gores GJ, et al. Biliary multifocal chromosomal polysomy and cholangiocarcinoma in primary sclerosing cholangitis. Am J Gastroenterol 2015;110:299–309
Bangarulingam SY, Bjornsson E, Enders F, et al. Long-term outcomes of positive fluorescence in situ hybridization tests in primary sclerosing cholangitis. Hepatology 2010;51:174–180
Barr Fritcher EG, Kipp BR, Voss JS, et al. Primary sclerosing cholangitis patients with serial polysomy fluorescence in situ hybridization results are at increased risk of cholangiocarcinoma. Am J Gastroenterol 2011;106:2023–2028
Dudley JC, Zheng Z, McDonald T, et al. Next-Generation sequencing and fluorescence in situ hybridization have comparable performance characteristics in the analysis of pancreaticobiliary brushings for malignancy. J Mol Diagn 2016;18:124–130
Tischendorf JJ, Kruger M, Trautwein C, et al. Cholangioscopic characterization of dominant bile duct stenoses in patients with primary sclerosing cholangitis. Endoscopy 2006; 38:665–669
Meining A, Chen YK, Pleskow D, et al. Direct visualization of indeterminate pancreaticobiliary strictures with probe-based confocal laser endomicroscopy: a multicenter experience. Gastrointest Endosc 2011;74:961–968
Slivka A, Gan I, Jamidar P, et al. Validation of the diagnostic accuracy of probe-based confocal laser endomicroscopy for the characterization of indeterminate biliary strictures: results of a prospective multicenter international study. Gastrointest Endosc 2015;81:282–290
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Jawad Ahmad declares that he has no competing interests.
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Ahmad, J. Metal, magnet or transplant: options in primary sclerosing cholangitis with stricture. Hepatol Int 12, 510–519 (2018). https://doi.org/10.1007/s12072-018-9906-6
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DOI: https://doi.org/10.1007/s12072-018-9906-6