Sclerosing Cholangitis: Clinicopathologic Features, Imaging Spectrum, and Systemic Approach to Differential Diagnosis

Overview

Journal Korean J Radiol

Specialty Radiology

Date 2016 Jan 23

PMID 26798213

Citations 20

Authors

Nieun Seo

So Yeon Kim

Seung Soo Lee

Jae Ho Byun

Jin Hee Kim

Hyoung Jung Kim

Moon-Gyu Lee

Affiliations

Soon will be listed here.

Abstract

Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

Citing Articles

Deep learning radiomics nomogram for preoperatively identifying moderate-to-severe chronic cholangitis in children with pancreaticobiliary maljunction: a multicenter study.

Mao H, Chen K, Zhu B, Guo W, Shi S BMC Med Imaging. 2025; 25(1):40.

PMID: 39910477 PMC: 11800502. DOI: 10.1186/s12880-025-01579-3.

Imaging of Benign Biliary Tract Disease.

Ghuman S, Buxi T, Jain K, Rawat K, Yadav A, Sud S Indian J Radiol Imaging. 2024; 34(4):726-739.

PMID: 39318553 PMC: 11419767. DOI: 10.1055/s-0044-1786038.

Primary sclerosing cholangitis: Is qualitative and quantitative 3 T MR imaging useful for the evaluation of disease severity?.

Boraschi P, Mazzantini V, Donati F, Coco B, Vianello B, Pinna A Eur J Radiol Open. 2024; 13:100595.

PMID: 39206437 PMC: 11357777. DOI: 10.1016/j.ejro.2024.100595.

Post-COVID-19 Cholangiopathy: Clinical and Radiologic Findings.

Kim H, Lee S Korean J Radiol. 2023; 24(11):1167-1171.

PMID: 37899526 PMC: 10613835. DOI: 10.3348/kjr.2023.0832.

Overlap syndrome of primary biliary cholangitis and primary sclerosing cholangitis: two case reports.

Yacoub H, Ben Azouz S, Hassine H, Debbabi H, Cherif D, Ghayeb F J Med Case Rep. 2023; 17(1):169.

PMID: 37106413 PMC: 10142480. DOI: 10.1186/s13256-023-03908-y.

References

Elsayes K, Oliveira E, Narra V, Abou El Abbass H, Ahmed M, Tongdee R . MR and MRCP in the evaluation of primary sclerosing cholangitis: current applications and imaging findings. J Comput Assist Tomogr. 2006; 30(3):398-404. DOI: 10.1097/00004728-200605000-00009. View

Karlsen T, Franke A, Melum E, Kaser A, Hov J, Balschun T . Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology. 2009; 138(3):1102-11. DOI: 10.1053/j.gastro.2009.11.046. View

Nashed C, Sakpal S, Shusharina V, Chamberlain R . Eosinophilic cholangitis and cholangiopathy: a sheep in wolves clothing. HPB Surg. 2010; 2010:906496. PMC: 2976516. DOI: 10.1155/2010/906496. View

Tsui W, Chan Y, Wong C, Lo Y, Yeung Y, Lee Y . Hepatolithiasis and the syndrome of recurrent pyogenic cholangitis: clinical, radiologic, and pathologic features. Semin Liver Dis. 2011; 31(1):33-48. DOI: 10.1055/s-0031-1272833. View

Shimosegawa T, Chari S, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M . International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011; 40(3):352-8. DOI: 10.1097/MPA.0b013e3182142fd2. View

Vlachou P, Khalili K, Jang H, Fischer S, Hirschfield G, Kim T . IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations. Radiographics. 2011; 31(5):1379-402. DOI: 10.1148/rg.315105735. View

Azizi L, Raynal M, Cazejust J, Ruiz A, Menu Y, Arrive L . MR Imaging of sclerosing cholangitis. Clin Res Hepatol Gastroenterol. 2012; 36(2):130-8. DOI: 10.1016/j.clinre.2011.11.011. View

Abdalian R, Heathcote E . Sclerosing cholangitis: a focus on secondary causes. Hepatology. 2006; 44(5):1063-74. DOI: 10.1002/hep.21405. View

Kim J, Kim T, Eun H, Byun J, Lee M, Ha H . CT findings of cholangiocarcinoma associated with recurrent pyogenic cholangitis. AJR Am J Roentgenol. 2006; 187(6):1571-7. DOI: 10.2214/AJR.05.0486. View

10.

Tischendorf J, Hecker H, Kruger M, Manns M, Meier P . Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study. Am J Gastroenterol. 2006; 102(1):107-14. DOI: 10.1111/j.1572-0241.2006.00872.x. View

11.

Nishino T, Oyama H, Hashimoto E, Toki F, Oi I, Kobayashi M . Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis. J Gastroenterol. 2007; 42(7):550-9. DOI: 10.1007/s00535-007-2038-8. View

12.

Kim J, Lee J, Han J, Kim S, Lee J, Choi J . Contrast-enhanced MRI combined with MR cholangiopancreatography for the evaluation of patients with biliary strictures: differentiation of malignant from benign bile duct strictures. J Magn Reson Imaging. 2007; 26(2):304-12. DOI: 10.1002/jmri.20973. View

13.

Kawamoto S, Siegelman S, Hruban R, Fishman E . Lymphoplasmacytic sclerosing pancreatitis (autoimmune pancreatitis): evaluation with multidetector CT. Radiographics. 2008; 28(1):157-70. DOI: 10.1148/rg.281065188. View

14.

Campsen J, Zimmerman M, Trotter J, Wachs M, Bak T, Steinberg T . Clinically recurrent primary sclerosing cholangitis following liver transplantation: a time course. Liver Transpl. 2008; 14(2):181-5. DOI: 10.1002/lt.21313. View

15.

Al-Sukhni W, Gallinger S, Pratzer A, Wei A, Ho C, Kortan P . Recurrent pyogenic cholangitis with hepatolithiasis--the role of surgical therapy in North America. J Gastrointest Surg. 2007; 12(3):496-503. DOI: 10.1007/s11605-007-0398-2. View

16.

Hoon Lim J, Mairiang E, Ahn G . Biliary parasitic diseases including clonorchiasis, opisthorchiasis and fascioliasis. Abdom Imaging. 2007; 33(2):157-65. DOI: 10.1007/s00261-007-9326-x. View

17.

Kim M, Cha S, Mitchell D, Chung J, Park S, Chung J . MR imaging findings in recurrent pyogenic cholangitis. AJR Am J Roentgenol. 1999; 173(6):1545-9. DOI: 10.2214/ajr.173.6.10584799. View

18.

Fulcher A, Turner M, Franklin K, Shiffman M, Sterling R, Luketic V . Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-control study. Radiology. 2001; 215(1):71-80. DOI: 10.1148/radiology.215.1.r00ap2671. View

19.

Vitellas K, Keogan M, Freed K, Enns R, Spritzer C, Baillie J . Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. Radiographics. 2000; 20(4):959-75; quiz 1108-9, 1112. DOI: 10.1148/radiographics.20.4.g00jl04959. View

20.

Saarinen S, Olerup O, Broome U . Increased frequency of autoimmune diseases in patients with primary sclerosing cholangitis. Am J Gastroenterol. 2000; 95(11):3195-9. DOI: 10.1111/j.1572-0241.2000.03292.x. View