Abstract
Background
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan. The aim of this study was to establish Japanese diagnostic criteria for PSC according to the current Japanese conditions.
Methods
The Intractable Hepato-Biliary Diseases Study Group in Japan of the Committee of Research on Measures for Intractable Diseases established a working group consisting of researchers specializing in PSC and proposed the 2016 Japanese diagnostic criteria for PSC.
Results
The diagnostic criteria consisted of the major and minor items. Major items are directly related to bile duct changes based on biliary tract imaging and an increased alkaline phosphatase level. Minor items consist of an association with inflammatory bowel disease and liver histology.
Conclusion
The diagnostic criteria for PSC are useful for general physicians in practice.
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References
Lazaridis KN, Wiesner RH, Porayko MK, et al. Primary sclerosing cholangitis. In: Schiff ER, Sorrel MF, Maddrey WC, editors. Schiff’s disease of the liver. English edition. Philadelphia: Lippincott-Raven; 1999. p. 649–78.
Lindor KD, LaRusso NF. Primary sclerosing cholangitis. In: Schiff L, Schiff ER, editors. Schiff’s disease of the liver. 9th ed. Philadelphia: JB Lippincott; 2003. p. 673–84.
Nakazawa T, Ando T, Hayashi K, et al. Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol. 2012;40:79–87.
Ohara H, Okazaki K, Tsubouchi H, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepato Biliary Pancreat Sci. 2012;19:536–42.
Takikawa H, Manabe T. Primary sclerosing cholangitis in Japan–analysis of 192 cases. J Gastroenterol. 1997;32:134–7.
Takikawa H, Takamori Y, Tanaka A, et al. Analysis of 388 cases of primary sclerosing cholangitis in Japan: presence of a subgroup without pancreatic involvement in older patients. Hepatol Res. 2004;29:153–9.
Tanaka A, Tazuma S, Okazaki K, et al. Nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan. J Hepato Biliary Pancreat Sci. 2014;21:43–50.
Tanaka A, Tazuma S, Okazaki K, et al. Clinical profiles of patients with primary sclerosing cholangitis in the elderly. J Hepato Biliary Pancreat Sci. 2015;22:230–6.
Nakazawa T, Naitoh I, Hayashi K, et al. Diagnosis of IgG4-related sclerosing cholangitis. World J Gastroenterol. 2013;19:7661–70.
Bjornsson E, Olsson R, Bergquist A, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology. 2008;134:975–80.
Hirschfield GM, Karlsen TH, Lindor KD, et al. Primary sclerosing cholangitis. Lancet. 2013;382:1587–99.
Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010;51:660–78.
Nakazawa T, Ohara H, Sano H, et al. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointest Endosc. 2004;60:937–44.
Kubota K, Kato S, Uchiyama T, et al. Discrimination between sclerosing cholangitis-associated autoimmune pancreatitis and primary sclerosing cholangitis, cancer using intraductal ultrasonography. Dig Endosc. 2011;23:10–6.
Naitoh I, Nakazawa T, Hayashi K, et al. Comparison of intraductal ultrasonography findings between primary sclerosing cholangitis and IgG4-related sclerosing cholangitis. J Gastroenterol Hepatol. 2015;30:1104–9.
Itoi T, Kamisawa T, Igarashi Y, et al. The role of peroral video cholangioscopy in patients with IgG4-related sclerosing cholangitis. J Gastroenterol. 2013;48:504–14.
Eaton JE, Talwalkar JA, Lazaridis KN, et al. Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management. Gastroenterology. 2013;145:521–36.
Nakazawa T, Naitoh I, Hayashi K, et al. Inflammatory bowel disease of primary sclerosing cholangitis: A distinct entity? World J Gastroenterol. 2014;20:3245–54.
Loftus EV, Harewood GC, Loftus CG, et al. PSCIBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut. 2005;54:91–6.
Boonstra K, van Erpecum KJ, van Nieuwkerk KM, et al. Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease. Inflamm Bowel Dis. 2012;18:2270–6.
Sano H, Nakazawa T, Ando T, et al. Clinical characteristics of inflammatory bowel disease associated with primary sclerosing cholangitis. J Hepato Biliary Pancreat Sci. 2011;18:154–61.
Jørgensen KK, Grzyb K, Lundin KE, et al. Inflammatory bowel disease in patients with primary sclerosing cholangitis: clinical characterization in liver transplanted and nontransplanted patients. Inflamm Bowel Dis. 2012;18:536–45.
Batts KP. Autoimmune and chronic cholestatic disorders of the liver. In: Odze RD, Goldblum JR, editors. Surgical Pathology of the GI tract, liver, biliary tract, and pancreas. 3rd ed. Philadelphia: Elsevier; 2015. p. 1262–84.
Nakanuma Y, Yamaguchi K, Ohta G, et al. Pathological features of hepatolithiasis in Japan. Hum Pathol. 1988;19:1181–6.
Torbenson MS. Biopsy interpretation of the liver. Philadelphia: Wolter’s Kluwer Health; 2015.
Lindor KD, Kowdley KV, Harrison E. ACG clinical guideline: primary sclerosing cholangitis. Am J Gastroenterol. 2015;110:646–59.
Acknowledgements
This study was conducted and supported by Health Labor Science Research Grants from Research on Measures for Intractable Diseases, the Intractable Hepato-Biliary Diseases Study Group in Japan.
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Nakazawa, T., Notohara, K., Tazuma, S. et al. The 2016 diagnostic criteria for primary sclerosing cholangitis. J Gastroenterol 52, 838–844 (2017). https://doi.org/10.1007/s00535-016-1286-x
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DOI: https://doi.org/10.1007/s00535-016-1286-x