Primary sclerosing cholangitis classification

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 may be classified according to the size of bile ducts involved, as well as its association with autoimmune hepatitis. The three main subtypes are the classic subtype, small ducts subtype, and primary sclerosing cholangitis associated with autoimmune hepatitis.

Classification

Primary sclerosing cholangitis may be divided into 3 subtypes, according to the type of ducts involved, as well as its association with autoimmune hepatitis:^[1]^[2]^[3]^[4]

Subtype	Ducts Involved	Diagnostic Procedure	Histopathology
Classic	Small and large bile ducts	MRCP, ERCP	Mixed inflammatory cells around bile ducts; nonspecific and nondiagnostic finding
Small duct	Small bile ducts only	Liver biopsy	Mixed inflammatory cells around bile ducts; nonspecific and nondiagnostic finding
Associated with autoimmune hepatitis	Small and large bile ducts	MRCP, ERCP	Lymphoplasmacytic infiltrate, associated with interface hepatitis

References

↑ Lazaridis KN, LaRusso NF (2016). “Primary Sclerosing Cholangitis”. N. Engl. J. Med. 375 (12): 1161–70. doi:10.1056/NEJMra1506330. PMID 27653566.
↑ Angulo P, Maor-Kendler Y, Lindor KD (2002). “Small-duct primary sclerosing cholangitis: a long-term follow-up study”. Hepatology. 35 (6): 1494–500. doi:10.1053/jhep.2002.33202. PMID 12029635.
↑ Feldstein AE, Perrault J, El-Youssif M, Lindor KD, Freese DK, Angulo P (2003). “Primary sclerosing cholangitis in children: a long-term follow-up study”. Hepatology. 38 (1): 210–7. doi:10.1053/jhep.2003.50289. PMID 12830004.
↑ Kaya M, Angulo P, Lindor KD (2000). “Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system”. J. Hepatol. 33 (4): 537–42. PMID 11059857.

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[pmid27653566-1] Lazaridis KN, LaRusso NF (2016). “Primary Sclerosing Cholangitis”. N. Engl. J. Med. 375 (12): 1161–70. doi:10.1056/NEJMra1506330. PMID 27653566.

[pmid12029635-2] Angulo P, Maor-Kendler Y, Lindor KD (2002). “Small-duct primary sclerosing cholangitis: a long-term follow-up study”. Hepatology. 35 (6): 1494–500. doi:10.1053/jhep.2002.33202. PMID 12029635.

[pmid12830004-3] Feldstein AE, Perrault J, El-Youssif M, Lindor KD, Freese DK, Angulo P (2003). “Primary sclerosing cholangitis in children: a long-term follow-up study”. Hepatology. 38 (1): 210–7. doi:10.1053/jhep.2003.50289. PMID 12830004.

[pmid11059857-4] Kaya M, Angulo P, Lindor KD (2000). “Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system”. J. Hepatol. 33 (4): 537–42. PMID 11059857.

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Primary sclerosing cholangitis classification

Overview

Classification

References

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