Primary sclerosing cholangitis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dima Nimri, M.D. [2]
Overview
Primary sclerosing cholangitis is a progressive disease that often results in death from complications of liver failure, liver transplantation or associated carcinomas. Even with liver transplantation, there is a substantial risk of recurrence of the disease. Complications of primary sclerosing cholangitis include osteoporosis, cholangiocarcinoma and liver failure.
Natural History
Primary sclerosing cholangitis is a progressive and often fatal disease. Death in patients with primary sclerosing cholangitis often results from complications of liver failure or associated cancers, such as cholangiocarcinoma, hepatocellular carcinoma, and colorectal carcinoma in patients with associated IBD.[1] The median survival from diagnosis to death in patients with primary sclerosing cholangitis ranges between 12-18 years.[2]
Complications
Some of the complications of primary sclerosing cholangitis include:[3]
- Osteoporosis:
- Liver cirrhosis and end stage liver disease (ESLD).
- Cholangiocarcinoma:
- The risk of cholangiocarcinoma in patients with primary sclerosing cholangitis is 400 times higher than the risk in the general population.[5]
Prognosis
Primary sclerosing cholangitis is a slowly progressive disease that may ultimately lead to liver failure.[3] Approximately 40% of patients require liver transplantation.[6] Even then, the 1 year survival rate after liver transplantation is 85% and 72% after 5 years. 25% of patients have recurrence of the disease after transplantation.[7]
The following factors are associated with a poorer prognosis in patients with primary sclerosing cholangitis:[3]
- The classic disease is associated with a poorer prognosis than small bile duct disease.[8][5]
- Patients with primary sclerosing cholangitis and an increased serum level of IgG4 have a worse prognosis than patients with primary sclerosing cholangitis and a normal serum level of IgG4.[9]
- After one to two years of diagnosis, patients with serum alkaline phosphatase level of ≥1.5 times the upper limit of the normal range have a worse outcome than patients who have serum alkaline phosphatase levels of less than 1.5 times the upper limit of the normal range.[10][11][12]
- High grade intrahepatic strictures on cholangiography.[2]
The Mayo Score for PSC uses an equation to predict the survival in patients with primary sclerosing cholangitis. The variables in this equation include age, AST levels, total bilirubin levels, serum albumin, as well as variceal bleeding.[13]
References
- ↑ Khaderi SA, Sussman NL (2015). "Screening for malignancy in primary sclerosing cholangitis (PSC)". Curr Gastroenterol Rep. 17 (4): 17. doi:10.1007/s11894-015-0438-0. PMID 25786901.
- ↑ 2.0 2.1 Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA, Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN (2002). "Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population". Gut. 51 (4): 562–6. PMC 1773389. PMID 12235081.
- ↑ 3.0 3.1 3.2 Lazaridis KN, LaRusso NF (2016). "Primary Sclerosing Cholangitis". N. Engl. J. Med. 375 (12): 1161–70. doi:10.1056/NEJMra1506330. PMID 27653566.
- ↑ Angulo P, Grandison GA, Fong DG, Keach JC, Lindor KD, Bjornsson E, Koch A (2011). "Bone disease in patients with primary sclerosing cholangitis". Gastroenterology. 140 (1): 180–8. doi:10.1053/j.gastro.2010.10.014. PMC 3043598. PMID 20955707.
- ↑ 5.0 5.1 Boonstra K, Weersma RK, van Erpecum KJ, Rauws EA, Spanier BW, Poen AC, van Nieuwkerk KM, Drenth JP, Witteman BJ, Tuynman HA, Naber AH, Kingma PJ, van Buuren HR, van Hoek B, Vleggaar FP, van Geloven N, Beuers U, Ponsioen CY (2013). "Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis". Hepatology. 58 (6): 2045–55. doi:10.1002/hep.26565. PMID 23775876.
- ↑ Tischendorf JJ, Hecker H, Krüger M, Manns MP, Meier PN (2007). "Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study". Am. J. Gastroenterol. 102 (1): 107–14. doi:10.1111/j.1572-0241.2006.00872.x. PMID 17037993.
- ↑ Fosby B, Karlsen TH, Melum E (2012). "Recurrence and rejection in liver transplantation for primary sclerosing cholangitis". World J. Gastroenterol. 18 (1): 1–15. doi:10.3748/wjg.v18.i1.1. PMC 3251800. PMID 22228965.
- ↑ Björnsson E, Olsson R, Bergquist A, Lindgren S, Braden B, Chapman RW, Boberg KM, Angulo P (2008). "The natural history of small-duct primary sclerosing cholangitis". Gastroenterology. 134 (4): 975–80. doi:10.1053/j.gastro.2008.01.042. PMID 18395078.
- ↑ Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, Chari S, Lindor KD (2006). "Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis". Am. J. Gastroenterol. 101 (9): 2070–5. doi:10.1111/j.1572-0241.2006.00772.x. PMID 16879434.
- ↑ Stanich PP, Björnsson E, Gossard AA, Enders F, Jorgensen R, Lindor KD (2011). "Alkaline phosphatase normalization is associated with better prognosis in primary sclerosing cholangitis". Dig Liver Dis. 43 (4): 309–13. doi:10.1016/j.dld.2010.12.008. PMC 3057302. PMID 21251891.
- ↑ Al Mamari S, Djordjevic J, Halliday JS, Chapman RW (2013). "Improvement of serum alkaline phosphatase to <1.5 upper limit of normal predicts better outcome and reduced risk of cholangiocarcinoma in primary sclerosing cholangitis". J. Hepatol. 58 (2): 329–34. doi:10.1016/j.jhep.2012.10.013. PMID 23085647.
- ↑ Rupp C, Rössler A, Halibasic E, Sauer P, Weiss KH, Friedrich K, Wannhoff A, Stiehl A, Stremmel W, Trauner M, Gotthardt DN (2014). "Reduction in alkaline phosphatase is associated with longer survival in primary sclerosing cholangitis, independent of dominant stenosis". Aliment. Pharmacol. Ther. 40 (11–12): 1292–301. doi:10.1111/apt.12979. PMID 25316001.
- ↑ Mayo Risk Score Calculator http://www.psc-literature.org/mrscalc.htm Accessed on Dec 12, 2016.