Primary sclerosing cholangitis natural history, complications and prognosis: Difference between revisions

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==Overview==
==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]].
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==
==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]].<ref name="pmid25786901">{{cite journal |vauthors=Khaderi SA, Sussman NL |title=Screening for malignancy in primary sclerosing cholangitis (PSC) |journal=Curr Gastroenterol Rep |volume=17 |issue=4 |pages=17 |year=2015 |pmid=25786901 |doi=10.1007/s11894-015-0438-0 |url=}}</ref> The median survival from diagnosis to death in patients with primary sclerosing cholangitis ranges between 12-18 years.<ref name="pmid12235081">{{cite journal |vauthors=Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA, Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN |title=Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population |journal=Gut |volume=51 |issue=4 |pages=562–6 |year=2002 |pmid=12235081 |pmc=1773389 |doi= |url=}}</ref>
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]].<ref name="pmid25786901">{{cite journal |vauthors=Khaderi SA, Sussman NL |title=Screening for malignancy in primary sclerosing cholangitis (PSC) |journal=Curr Gastroenterol Rep |volume=17 |issue=4 |pages=17 |year=2015 |pmid=25786901 |doi=10.1007/s11894-015-0438-0 |url=}}</ref> The [[median]] survival from [[diagnosis]] to death in patients with primary sclerosing cholangitis ranges between 12-18 years.<ref name="pmid12235081">{{cite journal |vauthors=Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA, Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN |title=Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population |journal=Gut |volume=51 |issue=4 |pages=562–6 |year=2002 |pmid=12235081 |pmc=1773389 |doi= |url=}}</ref>


==Complications==
==Complications==
Some of the complications of primary sclerosing cholangitis include:<ref name="pmid27653566">{{cite journal |vauthors=Lazaridis KN, LaRusso NF |title=Primary Sclerosing Cholangitis |journal=N. Engl. J. Med. |volume=375 |issue=12 |pages=1161–70 |year=2016 |pmid=27653566 |doi=10.1056/NEJMra1506330 |url=}}</ref>
Some of the [[complications]] of primary sclerosing cholangitis include:<ref name="pmid27653566">{{cite journal |vauthors=Lazaridis KN, LaRusso NF |title=Primary Sclerosing Cholangitis |journal=N. Engl. J. Med. |volume=375 |issue=12 |pages=1161–70 |year=2016 |pmid=27653566 |doi=10.1056/NEJMra1506330 |url=}}</ref>
*[[Osteoporosis]]: Patients with primary sclerosing cholangitis lose 1% of their bone mass every year.<ref name="pmid20955707">{{cite journal |vauthors=Angulo P, Grandison GA, Fong DG, Keach JC, Lindor KD, Bjornsson E, Koch A |title=Bone disease in patients with primary sclerosing cholangitis |journal=Gastroenterology |volume=140 |issue=1 |pages=180–8 |year=2011 |pmid=20955707 |pmc=3043598 |doi=10.1053/j.gastro.2010.10.014 |url=}}</ref>
*[[Osteoporosis]]:  
*[[Liver cirrhosis]] and [[end-stage liver disease]] ([[ESLD]]).
**Patients with primary sclerosing cholangitis lose 1% of their [[bone mass]] every year.<ref name="pmid20955707">{{cite journal |vauthors=Angulo P, Grandison GA, Fong DG, Keach JC, Lindor KD, Bjornsson E, Koch A |title=Bone disease in patients with primary sclerosing cholangitis |journal=Gastroenterology |volume=140 |issue=1 |pages=180–8 |year=2011 |pmid=20955707 |pmc=3043598 |doi=10.1053/j.gastro.2010.10.014 |url=}}</ref>
*[[Cholangiocarcinoma]]: the risk of [[cholangiocarcinoma]] in patients with primary sclerosing cholangitis is 400 times higher than the risk in the general population.<ref name="pmid23775876">{{cite journal |vauthors=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 |title=Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis |journal=Hepatology |volume=58 |issue=6 |pages=2045–55 |year=2013 |pmid=23775876 |doi=10.1002/hep.26565 |url=}}</ref>
*[[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.<ref name="pmid23775876">{{cite journal |vauthors=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 |title=Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis |journal=Hepatology |volume=58 |issue=6 |pages=2045–55 |year=2013 |pmid=23775876 |doi=10.1002/hep.26565 |url=}}</ref>


==Prognosis==
==Prognosis==


Primary sclerosing cholangitis is a slowly progressive disease that may ultimately lead to [[liver failure]].<ref name="pmid27653566">{{cite journal |vauthors=Lazaridis KN, LaRusso NF |title=Primary Sclerosing Cholangitis |journal=N. Engl. J. Med. |volume=375 |issue=12 |pages=1161–70 |year=2016 |pmid=27653566 |doi=10.1056/NEJMra1506330 |url=}}</ref> Approximately 40% of patients require [[liver transplantation]].<ref name="pmid17037993">{{cite journal |vauthors=Tischendorf JJ, Hecker H, Krüger M, Manns MP, Meier PN |title=Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study |journal=Am. J. Gastroenterol. |volume=102 |issue=1 |pages=107–14 |year=2007 |pmid=17037993 |doi=10.1111/j.1572-0241.2006.00872.x |url=}}</ref> 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.<ref name="pmid22228965">{{cite journal |vauthors=Fosby B, Karlsen TH, Melum E |title=Recurrence and rejection in liver transplantation for primary sclerosing cholangitis |journal=World J. Gastroenterol. |volume=18 |issue=1 |pages=1–15 |year=2012 |pmid=22228965 |pmc=3251800 |doi=10.3748/wjg.v18.i1.1 |url=}}</ref>  
Primary sclerosing cholangitis is a slowly progressive [[disease]] that may ultimately lead to [[liver failure]].<ref name="pmid27653566">{{cite journal |vauthors=Lazaridis KN, LaRusso NF |title=Primary Sclerosing Cholangitis |journal=N. Engl. J. Med. |volume=375 |issue=12 |pages=1161–70 |year=2016 |pmid=27653566 |doi=10.1056/NEJMra1506330 |url=}}</ref> Approximately 40% of patients require [[liver transplantation]].<ref name="pmid17037993">{{cite journal |vauthors=Tischendorf JJ, Hecker H, Krüger M, Manns MP, Meier PN |title=Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study |journal=Am. J. Gastroenterol. |volume=102 |issue=1 |pages=107–14 |year=2007 |pmid=17037993 |doi=10.1111/j.1572-0241.2006.00872.x |url=}}</ref> 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]].<ref name="pmid22228965">{{cite journal |vauthors=Fosby B, Karlsen TH, Melum E |title=Recurrence and rejection in liver transplantation for primary sclerosing cholangitis |journal=World J. Gastroenterol. |volume=18 |issue=1 |pages=1–15 |year=2012 |pmid=22228965 |pmc=3251800 |doi=10.3748/wjg.v18.i1.1 |url=}}</ref>  


The following factors are associated with a poorer prognosis in patients with primary sclerosing cholangitis:<ref name="pmid27653566">{{cite journal |vauthors=Lazaridis KN, LaRusso NF |title=Primary Sclerosing Cholangitis |journal=N. Engl. J. Med. |volume=375 |issue=12 |pages=1161–70 |year=2016 |pmid=27653566 |doi=10.1056/NEJMra1506330 |url=}}</ref>  
The following factors are associated with a poorer [[prognosis]] in patients with primary sclerosing cholangitis:<ref name="pmid27653566">{{cite journal |vauthors=Lazaridis KN, LaRusso NF |title=Primary Sclerosing Cholangitis |journal=N. Engl. J. Med. |volume=375 |issue=12 |pages=1161–70 |year=2016 |pmid=27653566 |doi=10.1056/NEJMra1506330 |url=}}</ref>  
*The classic disease is associated with a poorer prognosis than small [[bile duct]] disease.<ref name="pmid18395078">{{cite journal |vauthors=Björnsson E, Olsson R, Bergquist A, Lindgren S, Braden B, Chapman RW, Boberg KM, Angulo P |title=The natural history of small-duct primary sclerosing cholangitis |journal=Gastroenterology |volume=134 |issue=4 |pages=975–80 |year=2008 |pmid=18395078 |doi=10.1053/j.gastro.2008.01.042 |url=}}</ref><ref name="pmid23775876">{{cite journal |vauthors=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 |title=Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis |journal=Hepatology |volume=58 |issue=6 |pages=2045–55 |year=2013 |pmid=23775876 |doi=10.1002/hep.26565 |url=}}</ref>
*The classic disease is associated with a poorer [[prognosis]] than small [[bile duct]] disease.<ref name="pmid18395078">{{cite journal |vauthors=Björnsson E, Olsson R, Bergquist A, Lindgren S, Braden B, Chapman RW, Boberg KM, Angulo P |title=The natural history of small-duct primary sclerosing cholangitis |journal=Gastroenterology |volume=134 |issue=4 |pages=975–80 |year=2008 |pmid=18395078 |doi=10.1053/j.gastro.2008.01.042 |url=}}</ref><ref name="pmid23775876">{{cite journal |vauthors=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 |title=Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis |journal=Hepatology |volume=58 |issue=6 |pages=2045–55 |year=2013 |pmid=23775876 |doi=10.1002/hep.26565 |url=}}</ref>
*Patients with primary sclerosing cholangitis and an increased [[serum]] level of [[IgG]]4 have a worse prognosis than patients with primary sclerosing cholangitis and a normal [[serum]] level of [[IgG]]4.<ref name="pmid16879434">{{cite journal |vauthors=Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, Chari S, Lindor KD |title=Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis |journal=Am. J. Gastroenterol. |volume=101 |issue=9 |pages=2070–5 |year=2006 |pmid=16879434 |doi=10.1111/j.1572-0241.2006.00772.x |url=}}</ref>
*Patients with primary sclerosing cholangitis and an increased [[serum]] level of [[IgG]]4 have a worse [[prognosis]] than patients with primary sclerosing cholangitis and a normal [[serum]] level of [[IgG]]4.<ref name="pmid16879434">{{cite journal |vauthors=Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, Chari S, Lindor KD |title=Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis |journal=Am. J. Gastroenterol. |volume=101 |issue=9 |pages=2070–5 |year=2006 |pmid=16879434 |doi=10.1111/j.1572-0241.2006.00772.x |url=}}</ref>
*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.<ref name="pmid21251891">{{cite journal |vauthors=Stanich PP, Björnsson E, Gossard AA, Enders F, Jorgensen R, Lindor KD |title=Alkaline phosphatase normalization is associated with better prognosis in primary sclerosing cholangitis |journal=Dig Liver Dis |volume=43 |issue=4 |pages=309–13 |year=2011 |pmid=21251891 |pmc=3057302 |doi=10.1016/j.dld.2010.12.008 |url=}}</ref><ref name="pmid23085647">{{cite journal |vauthors=Al Mamari S, Djordjevic J, Halliday JS, Chapman RW |title=Improvement of serum alkaline phosphatase to <1.5 upper limit of normal predicts better outcome and reduced risk of cholangiocarcinoma in primary sclerosing cholangitis |journal=J. Hepatol. |volume=58 |issue=2 |pages=329–34 |year=2013 |pmid=23085647 |doi=10.1016/j.jhep.2012.10.013 |url=}}</ref><ref name="pmid25316001">{{cite journal |vauthors=Rupp C, Rössler A, Halibasic E, Sauer P, Weiss KH, Friedrich K, Wannhoff A, Stiehl A, Stremmel W, Trauner M, Gotthardt DN |title=Reduction in alkaline phosphatase is associated with longer survival in primary sclerosing cholangitis, independent of dominant stenosis |journal=Aliment. Pharmacol. Ther. |volume=40 |issue=11-12 |pages=1292–301 |year=2014 |pmid=25316001 |doi=10.1111/apt.12979 |url=}}</ref>
*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.<ref name="pmid21251891">{{cite journal |vauthors=Stanich PP, Björnsson E, Gossard AA, Enders F, Jorgensen R, Lindor KD |title=Alkaline phosphatase normalization is associated with better prognosis in primary sclerosing cholangitis |journal=Dig Liver Dis |volume=43 |issue=4 |pages=309–13 |year=2011 |pmid=21251891 |pmc=3057302 |doi=10.1016/j.dld.2010.12.008 |url=}}</ref><ref name="pmid23085647">{{cite journal |vauthors=Al Mamari S, Djordjevic J, Halliday JS, Chapman RW |title=Improvement of serum alkaline phosphatase to <1.5 upper limit of normal predicts better outcome and reduced risk of cholangiocarcinoma in primary sclerosing cholangitis |journal=J. Hepatol. |volume=58 |issue=2 |pages=329–34 |year=2013 |pmid=23085647 |doi=10.1016/j.jhep.2012.10.013 |url=}}</ref><ref name="pmid25316001">{{cite journal |vauthors=Rupp C, Rössler A, Halibasic E, Sauer P, Weiss KH, Friedrich K, Wannhoff A, Stiehl A, Stremmel W, Trauner M, Gotthardt DN |title=Reduction in alkaline phosphatase is associated with longer survival in primary sclerosing cholangitis, independent of dominant stenosis |journal=Aliment. Pharmacol. Ther. |volume=40 |issue=11-12 |pages=1292–301 |year=2014 |pmid=25316001 |doi=10.1111/apt.12979 |url=}}</ref>
*High grade intrahepatic [[strictures]] on cholangiography.<ref name="pmid12235081">{{cite journal |vauthors=Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA, Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN |title=Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population |journal=Gut |volume=51 |issue=4 |pages=562–6 |year=2002 |pmid=12235081 |pmc=1773389 |doi= |url=}}</ref><br>
*High grade intrahepatic [[strictures]] on cholangiography.<ref name="pmid12235081">{{cite journal |vauthors=Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA, Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN |title=Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population |journal=Gut |volume=51 |issue=4 |pages=562–6 |year=2002 |pmid=12235081 |pmc=1773389 |doi= |url=}}</ref><br>


''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.<ref name="Mayo Risk Score Calculator"> Mayo Risk Score Calculator http://www.psc-literature.org/mrscalc.htm Accessed on Dec 12, 2016. </ref>
''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.<ref name="Mayo Risk Score Calculator">Mayo Risk Score Calculator http://www.psc-literature.org/mrscalc.htm Accessed on Dec 12, 2016. </ref>


==References==
==References==

Latest revision as of 16:25, 6 December 2017

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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]

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 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

  1. ↑ 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. ↑ 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. ↑ 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.
  4. ↑ 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. ↑ 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ Mayo Risk Score Calculator http://www.psc-literature.org/mrscalc.htm Accessed on Dec 12, 2016.


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