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
Natural History
Complications
Some of the complications of primary sclerosing cholangitis include:[1]
- Osteoporosis: Patients with primary sclerosing cholangitis lose 1% of their bone mass every year.[2]
- 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.[3]
Prognosis
The following factors are associated with a poorer prognosis in patients with primary sclerosing cholangitis:[1]
- The classic disease is associated with a poorer prognosis than small bile duct disease.[4][3]
- 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.[5]
- 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.[6][7][8]
References
- ↑ 1.0 1.1 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.
- ↑ 3.0 3.1 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.
- ↑ 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.