Top

Published in:

Open Access 01-12-2023 | Primary Biliary Cholangitis | Critical Review

CT and MR imaging of primary biliary cholangitis: a pictorial review

Authors: Yun Zhang, Tianying Zheng, Zixing Huang, Bin Song

Published in: Insights into Imaging | Issue 1/2023

Abstract

Primary biliary cholangitis (PBC) is a rare chronic autoimmune-mediated cholestatic liver disease involving medium and small bile ducts that can lead to liver fibrosis and cirrhosis. To date, the pathogenesis of PBC remains elusive, and there is currently no curative medical treatment. Computed tomography (CT) and magnetic resonance (MR) imaging, as common technical tools that allow non-invasive monitoring of liver tissue in vivo, play crucial roles in the diagnosis, staging, and prognosis prediction in PBC by enabling assessment of abnormalities in liver morphology and parenchyma, irregular configuration of bile ducts, lymphadenopathy, portal hypertension, and complications of cirrhosis. Moreover, CT and MRI can be used to monitor the disease progression after treatment of PBC (e.g. the onset of cirrhotic decompensation or HCC) to guide the clinical decisions for liver transplantation. With the optimization of imaging technology, magnetic resonance elastography (MRE) offers additional information on liver stiffness, allows for the identification of early cirrhosis in PBC and provides a basis for predicting prognosis. Gadoxetic acid-enhanced MRI enables the assessment of liver function in patients with PBC. The purpose of this review is to detail and illustrate the definition, pathological basis, and clinical importance of CT and MRI features of PBC to help radiologists and clinicians enhance their understanding of PBC.

Critical Relevance Statement

Characteristic CT and MR imaging manifestations of primary biliary cholangitis may reflect the course of the disease and provide information associated with histological grading and altered cellular function.

Key points

• Imaging has become highly useful for differentiating PBC from other diseases.

• Key pathological alterations of PBC can be captured by CT and MRI.

• Characteristic manifestations provide information associated with histological grade and cellular function.

• Despite this, the CT or MRI features of PBC are not specific.

Graphical Abstract

Available only for authorised users

European Association for the Study of the Liver (2017) EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol 67(1):145–172

Wijarnpreecha K, Werlang M, Panjawatanan P et al (2019) Association between smoking and risk of primary biliary cholangitis: a systematic review and meta-analysis. J Gastrointestin Liver Dis 28:197–203PubMed

Ala A, Stanca CM, Bu-Ghanim M et al (2006) Increased prevalence of primary biliary cirrhosis near superfund toxic waste sites. Hepatology 43:525–531

Prince MI, Ducker SJ, James OF (2010) Case-control studies of risk factors for primary biliary cirrhosis in two united kingdom populations. Gut 59:508–512PubMed

Gulamhusein AF, Juran BD, Lazaridis KN (2015) Genome-wide association studies in primary biliary cirrhosis. Semin Liver Dis 35:392–401PubMedPubMedCentral

Cordell HJ, Fryett JJ, Ueno K et al (2021) An international genome-wide meta-analysis of primary biliary cholangitis: novel risk loci and candidate drugs. J Hepatol 75:572–581PubMedPubMedCentral

Selmi C, Lleo A, Pasini S, Zuin M, Gershwin ME (2009) Innate immunity and primary biliary cirrhosis. Curr Mol Med 9:45–51PubMed

Hirschfield GM, Gershwin ME (2013) The immunobiology and pathophysiology of primary biliary cirrhosis. Annu Rev Pathol 8:303–330PubMed

Prince MI, Chetwynd A, Craig WL, Metcalf JV, James OF (2004) Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population based cohort. Gut 53:865–870PubMedPubMedCentral

10.

Murillo Perez CF, Goet JC (2018) Milder disease stage in patients with primary biliary cholangitis over a 44-year period: a changing natural history. Hepatology 67:1920–1930PubMed

11.

You H, Ma X, Efe C et al (2022) APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis. Hepatol Int 16:1–23PubMed

12.

Carbone M, Mells GF, Pells G et al (2013) Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology 144:560–569 e567; quiz e513–564

13.

Trivedi PJ, Lammers WJ, van Buuren HR et al (2016) Stratification of hepatocellular carcinoma risk in primary biliary cirrhosis: a multicentre international study. Gut 65:321–329PubMed

14.

Ahrens EH Jr, Kunkel HG (1949) The relationship between serum lipids and skin xanthomata in 18 patients with primary biliary cirrhosis. J Clin Investig 28:1565–1574PubMedPubMedCentral

15.

Sherlock S (1959) Primary billiary cirrhosis (chronic intrahepatic obstructive jaundice). Gastroenterology 37:574–586PubMed

16.

Walker JG, Doniach D, Roitt IM, Sherlock S (1965) Serological tests in diagnosis of primary biliary cirrhosis. Lancet 1:827–831PubMed

17.

Beuers U, Gershwin ME, Gish RG et al (2015) Changing nomenclature for pbc: from 'cirrhosis' to 'cholangitis'. Hepatology 62:1620–1622

18.

Selmi C, Bowlus CL, Gershwin ME, Coppel RL (2011) Primary biliary cirrhosis. Lancet 377:1600–1609PubMed

19.

Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M (2019) Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology 69:394–419

20.

Hirschfield GM, Dyson JK, Alexander GJM et al (2018) The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut 67:1568–1594PubMed

21.

Kim KA, Ki M, Choi HY, Kim BH, Jang ES, Jeong SH (2016) Population-based epidemiology of primary biliary cirrhosis in South Korea. Aliment Pharmacol Ther 43:154–162PubMed

22.

Tanaka A, Mori M, Matsumoto K, Ohira H, Tazuma S, Takikawa H (2019) Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan. Hepatol Res 49:881–889PubMed

23.

Cheung KS, Seto WK, Fung J, Lai CL, Yuen MF (2017) Epidemiology and natural history of primary biliary cholangitis in the Chinese: a territory-based study in Hong Kong between 2000 and 2015. Clin Transl Gastroenterol 8:e116PubMedPubMedCentral

24.

Chen S, Duan W, Li M et al (2019) Prognosis of 732 ursodeoxycholic acid-treated patients with primary biliary cholangitis: a single center follow-up study from China. J Gastroenterol Hepatol 34:1236–1241PubMed

25.

Lin CY, Cheng YT, Chang ML, Chien RN (2019) The extrahepatic events of Asian patients with primary biliary cholangitis: a 30-year cohort study. Sci Rep 9:7577PubMedPubMedCentral

26.

Lleo A, Colapietro F (2018) Changes in the epidemiology of primary biliary cholangitis. Clin Liver Dis 22:429–441PubMed

27.

Corpechot C, Chazouillères O, Poupon R (2011) Early primary biliary cirrhosis: Biochemical response to treatment and prediction of long-term outcome. J Hepatol 55:1361–1367PubMed

28.

Harms MH, van Buuren HR, Corpechot C et al (2019) Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis. J Hepatol 71:357–365PubMed

29.

Sarcognato S, Sacchi D, Grillo F et al (2021) Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis. Pathologica 113:170–184PubMedPubMedCentral

30.

Leung KK, Deeb M, Hirschfield GM (2020) Review article: pathophysiology and management of primary biliary cholangitis. Aliment Pharmacol Ther 52:1150–1164PubMed

31.

Ludwig J, Dickson ER, McDonald GS (1978) Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis). Virchows Archiv. A, Pathol Anat Histol 379:103–112

32.

Scheuer PJ (1983) Primary biliary cirrhosis: diagnosis, pathology and pathogenesis. Postgrad Med J 59(Suppl 4):106–115PubMed

33.

Nakanuma Y, Zen Y, Harada K et al (2010) Application of a new histological staging and grading system for primary biliary cirrhosis to liver biopsy specimens: Interobserver agreement. Pathol Int 60:167–174PubMed

34.

Kakuda Y, Harada K, Sawada-Kitamura S et al (2013) Evaluation of a new histologic staging and grading system for primary biliary cirrhosis in comparison with classical systems. Hum Pathol 44:1107–1117PubMed

35.

Wendum D, Boëlle PY, Bedossa P et al (2015) Primary biliary cirrhosis: proposal for a new simple histological scoring system. Liver Int 35:652–659PubMed

36.

Morgan MA, Sundaram KM (2023) Primary biliary cholangitis: review for radiologists. Abdom Radiol (NY) 48:127–135PubMed

37.

Kovač JD, Ješić R, Stanisavljević D et al (2012) Integrative role of MRI in the evaluation of primary biliary cirrhosis. Eur Radiol 22:688–694PubMed

38.

Idilman IS, Venkatesh SH, Eaton JE et al (2020) Magnetic resonance imaging features in 283 patients with primary biliary cholangitis. Eur Radiol 30:5139–5148PubMed

39.

Dodd GD 3rd, Baron RL, Oliver JH 3rd, Federle MP (1999) Spectrum of imaging findings of the liver in end-stage cirrhosis: part i, gross morphology and diffuse abnormalities. AJR Am J Roentgenol 173:1031–1036PubMed

40.

Ito K, Mitchell DG, Gabata T (2000) Enlargement of hilar periportal space: a sign of early cirrhosis at MR imaging. J Magn Reson Imaging 11:136–140PubMed

41.

Meng Y, Liang Y, Liu M (2015) The value of MRI in the diagnosis of primary biliary cirrhosis and assessment of liver fibrosis. PLoS One 10:e0120110

42.

Wenzel JS, Donohoe A, Ford KL 3rd, Glastad K, Watkins D, Molmenti E (2001) Primary biliary cirrhosis: MR imaging findings and description of MR imaging periportal halo sign. AJR Am J Roentgenol 176:885–889PubMed

43.

Haliloglu N, Erden A, Erden I (2009) Primary biliary cirrhosis: Evaluation with t2-weighted MR imaging and MR cholangiopancreatography. Eur J Radiol 69:523–527PubMed

44.

Blachar A, Federle MP, Brancatelli G (2001) Primary biliary cirrhosis: clinical, pathologic, and helical ct findings in 53 patients. Radiology 220:329–336PubMed

45.

Kobayashi S, Matsui O, Gabata T et al (2005) Mri findings of primary biliary cirrhosis: correlation with Scheuer histologic staging. Abdom Imaging 30:71–76PubMed

46.

Guglielmo FF, Venkatesh SK, Mitchell DG (2019) Liver MR elastography technique and image interpretation: pearls and pitfalls. Radiographics 39:1983–2002

47.

Nilsson H, Blomqvist L, Douglas L, Nordell A, Jonas E (2010) Assessment of liver function in primary biliary cirrhosis using Gd-EOB-DTPA-enhanced liver MRI. HPB (Oxford) 12:567–576PubMed

48.

Han D, Liu J, Jin E, He W (2019) Liver assessment using Gd-EOB-DTPA-enhanced magnetic resonance imaging in primary biliary cholangitis patients. Jpn J Radiol 37:412–419PubMed

49.

Perez AA, Noe-Kim V, Lubner MG et al (2022) Deep learning ct-based quantitative visualization tool for liver volume estimation: defining normal and hepatomegaly. Radiology 302:336–342PubMed

50.

Kaplan MM, Gershwin ME (2005) Primary biliary cirrhosis. N Engl J Med 353:1261–1273PubMed

51.

Malik N, Venkatesh SK (2017) Imaging of autoimmune hepatitis and overlap syndromes. Abdom Radiol (NY) 42:19–27PubMed

52.

Takeyama Y, Tsuchiya N, Kunimoto H et al (2015) Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging as a useful detection method for advanced primary biliary cirrhosis. Hepatol Res 45:E108–114PubMed

53.

Navasa M, Parés A, Bruguera M, Caballería J, Bosch J, Rodés J (1987) Portal hypertension in primary biliary cirrhosis. Relationship with histological features. J Hepatol 5:292–298

54.

Huet PM, Vincent C, Deslaurier J et al (2008) Portal hypertension and primary biliary cirrhosis: effect of long-term ursodeoxycholic acid treatment. Gastroenterology 135:1552–1560PubMed

55.

Burghart L, Halilbasic E, Schwabl P et al (2022) Distinct prognostic value of different portal hypertension-associated features in patients with primary biliary cholangitis. J Gastroenterol 57:99–110PubMed

56.

Natarajan Y, Tansel A, Patel P et al (2021) Incidence of hepatocellular carcinoma in primary biliary cholangitis: a systematic review and meta-analysis. Dig Dis Sci 66:2439–2451PubMed

57.

Chernyak V, Fowler KJ, Kamaya A, Kielar AZ, Elsayes KM, Bashir MR (2018) Liver imaging reporting and data system (li-rads) version 2018: imaging of hepatocellular carcinoma in at-risk patients. Radiology 289:816–830PubMed

58.

Gatos I, Drazinos P, Yarmenitis S, Theotokas I, Zoumpoulis PS (2020) Comparison of sound touch elastography, shear wave elastography and vibration-controlled transient elastography in chronic liver disease assessment using liver biopsy as the “reference standard.” Ultrasound Med Biol 46:959–971PubMed

59.

Yang L, Ling W, He D et al (2021) Shear wave-based sound touch elastography in liver fibrosis assessment for patients with autoimmune liver diseases. Quant Imaging Med Surg 11:1532–1542PubMedPubMedCentral

60.

Gatos I, Yarmenitis S, Theotokas I et al (2022) Comparison of visual transient elastography, vibration controlled transient elastography, shear wave elastography and sound touch elastography in chronic liver disease assessment using liver biopsy as “gold standard.” Eur J Radiol 157:110557PubMed

61.

Guo Y, Parthasarathy S, Goyal P, McCarthy RJ, Larson AC, Miller FH (2015) Magnetic resonance elastography and acoustic radiation force impulse for staging hepatic fibrosis: a meta-analysis. Abdom Imaging 40:818–834PubMed

62.

Osman KT, Maselli DB, Idilman IS et al (2021) Liver stiffness measured by either magnetic resonance or transient elastography is associated with liver fibrosis and is an independent predictor of outcomes among patients with primary biliary cholangitis. J Clin Gastroenterol 55:449–457PubMedPubMedCentral

63.

Renzulli M, Biselli M, Brocchi S et al (2018) New hallmark of hepatocellular carcinoma, early hepatocellular carcinoma and high-grade dysplastic nodules on Gd-EOB-DTPA MRI in patients with cirrhosis: a new diagnostic algorithm. Gut 67:1674–1682PubMed

64.

Liu C, Sun Y, Yang Y et al (2021) Gadobenate dimeglumine-enhanced biliary imaging from the hepatobiliary phase can predict progression in patients with liver cirrhosis. Eur Radiol 31:5840–5850PubMed

65.

Vernuccio F, Cannella R, Gozzo C et al (2020) Hepatic enhancement in cirrhosis in the portal venous phase: what are the differences between gadoxetate disodium and gadobenate dimeglumine? Abdom Radiol (NY) 45:2409–2417PubMed

66.

KhouriChalouhi C, Vernuccio F, Rini F et al (2019) Hepatobiliary phase in cirrhotic patients with different model for end-stage liver disease score: Comparison of the performance of gadoxetic acid to gadobenate dimeglumine. Eur Radiol 29:3090–3099

67.

Bastati N, Beer L, Mandorfer M et al (2020) Does the functional liver imaging score derived from gadoxetic acid-enhanced MRI predict outcomes in chronic liver disease? Radiology 294:98–107PubMed

68.

Dalekos GN, Gatselis NK (2019) Variant and specific forms of autoimmune cholestatic liver diseases. Arch Immunol Ther Exp 67:197–211

69.

Boberg KM, Chapman RW, Hirschfield GM, Lohse AW, Manns MP, Schrumpf E (2011) Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. J Hepatol 54:374–385PubMed

70.

Dalekos GN, Koskinas J, Papatheodoridis GV (2019) Hellenic association for the study of the liver clinical practice guidelines: autoimmune hepatitis. Ann Gastroenterol 32:1–23PubMed

71.

Goertz RS, GaBmann L, Strobel D et al (2019) Acoustic radiation force impulse (ARFI) elastography in autoimmune and cholestatic liver diseases. Ann Hepatol 18:23–29PubMed

72.

Sun LL, Dong G, Wang B, Zheng Q, Wang S, Zhang RF (2016) Real-time shear wave elastrography and apri index for evaluating autoimmune hepatitis fibrosis. J Biol Regul Homeost Agents 30:1019–1021PubMed

73.

Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M (2018) Primary sclerosing cholangitis. Lancet 391:2547–2559PubMed

74.

Karlsen TH, Franke A, Melum E et al (2010) Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology 138:1102–1111PubMed

75.

Khoshpouri P, Habibabadi RR, Hazhirkarzar B, Ameli S, Ghadimi M, Ghasabeh MA (2019) Imaging features of primary sclerosing cholangitis: from diagnosis to liver transplant follow-up. Radiographics 39:1938–1964

76.

Venkatesh SK, Welle CL, Miller FH et al (2022) Reporting standards for primary sclerosing cholangitis using MRI and MR cholangiopancreatography: guidelines from MR Working Group of the International Primary Sclerosing Cholangitis Study Group. Eur Radiol 32:923–937PubMed

77.

Martínez-de-Alegría A, Baleato-González S, García-Figueiras R et al (2015) Igg4-related disease from head to toe. Radiographics 35:2007–2025PubMed

78.

Guidry C, Fricke RG, Ram R, Pandey T, Jambhekar K (2016) Imaging of sarcoidosis: a contemporary review. Radiol Clin North Am 54:519–534PubMed

79.

Lampert F (1998) Langerhans cell histiocytosis. Historical perspectives. Hematol/Oncol Clin North Am 12:213–219

80.

Murakami M, Onishi S, Ohya Y et al (2020) Langerhans cell histiocytosis confined to extrahepatic bile duct causing sclerosing cholangitis in child: a case report. Surg Case Rep 6:137PubMedPubMedCentral

81.

Aldarwish S, Schafmayer C, Erbersdobler A, Hinz S (2022) An extremely rare cause of an obstructive jaundice in adults: limited Langerhans cell histiocytosis of the extrahepatic bile duct. Visc Med 38:295–299PubMedPubMedCentral

82.

Consing M, Lee HE, Jess H, Vahidi S (2022) Solitary involvement of the liver: a rare manifestation of Langerhans cell histiocytosis. Am J Case Rep 23:e937628PubMedPubMedCentral

83.

O’Connor OJ, O’Neill S, Maher MM (2011) Imaging of biliary tract disease. AJR Am J Roentgenol 197:W551–558PubMed

84.

Corpechot C, Abenavoli L, Rabahi N et al (2008) Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 48:871–877

Title: CT and MR imaging of primary biliary cholangitis: a pictorial review
Authors: Yun Zhang
Tianying Zheng
Zixing Huang
Bin Song
Publication date: 01-12-2023
Publisher: Springer Vienna
Keywords: Primary Biliary Cholangitis
Computed Tomography
Computed Tomography
Magnetic Resonance Imaging
Magnetic Resonance Imaging
Primary Sclerosing Cholangitis
Magnetic Resonance Cholangio Pancreatography
Published in: Insights into Imaging / Issue 1/2023
Electronic ISSN: 1869-4101
DOI: https://doi.org/10.1186/s13244-023-01517-3

At a glance: The STEP trials

Springer Medicine

CT and MR imaging of primary biliary cholangitis: a pictorial review

Abstract

Graphical Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

Graphical Abstract

Please log in to get access to this content

Other articles of this Issue 1/2023

Multi-channel deep learning model-based myocardial spatial–temporal morphology feature on cardiac MRI cine images diagnoses the cause of LVH

Development of a simplified model and nomogram in preoperative diagnosis of pediatric chronic cholangitis with pancreaticobiliary maljunction using clinical variables and MRI radiomics

Methodological considerations on segmenting rhabdomyosarcoma with diffusion-weighted imaging—What can we do better?

Implementation of structured reporting in clinical routine: a review of 7 years of institutional experience

Clinicopathological and prognostic value of calcification morphology descriptors in ductal carcinoma in situ of the breast: a systematic review and meta-analysis

A deep learning model using chest X-ray for identifying TB and NTM-LD patients: a cross-sectional study