Non-neoplastic cholangiopathies: an algorithmic approach

Cholangiopathies are chronic diseases that affect the bile ducts, comprising a heterogeneous group of progressive and potentially fatal entities. The diagnosis of these diseases is a great challenge for radiologists because of the overlapping of their clinical, biochemical, and imaging findings. Nevertheless, identifying the precise etiology is crucial, given that the therapeutic options are distinct and influence the prognosis of the patient. The purpose of this review article is to discuss some of the non-neoplastic causes of cholangiopathies and to provide a useful diagnostic algorithm.

Keywords:
Bile duct diseases; Liver cirrhosis; Cholangiography; Computed tomography; Magnetic resonance imaging

Authorship

Marina Silva Zacarias Correspondence: Dra. Marina Silva Zacarias. EPM-Unifesp - Departamento de Diagnóstico por Imagem. Rua Napoleão de Barros, 800, Vila Clementino. São Paulo, SP, Brazil, 04023-062. Email: marina_sz@hotmail.com.

Departamento de Diagnóstico por Imagem - Escola Paulista de Medicina da Universidade Federal de São Paulo (EPM-Unifesp), São Paulo, SP, Brazil.Universidade Federal de São PauloBrazilSão Paulo, SP, BrazilDepartamento de Diagnóstico por Imagem - Escola Paulista de Medicina da Universidade Federal de São Paulo (EPM-Unifesp), São Paulo, SP, Brazil.

a https://orcid.org/0000-0001-8553-974X

https://orcid.org/0000-0001-8553-974X

Hanna Rafaela Ferreira Dalla Pria

b https://orcid.org/0000-0002-6556-7003

https://orcid.org/0000-0002-6556-7003

Rafael Andrade Santiago de Oliveira

c https://orcid.org/0000-0003-0838-3731

https://orcid.org/0000-0003-0838-3731

Luis Fernando Delmonte

d https://orcid.org/0000-0002-8601-4774

https://orcid.org/0000-0002-8601-4774

Fernanda Garozzo Velloni

e https://orcid.org/0000-0002-2019-7918

https://orcid.org/0000-0002-2019-7918

Giuseppe D’Ippolito

f https://orcid.org/0000-0002-2701-1928.

https://orcid.org/0000-0002-2701-1928

Correspondence: Dra. Marina Silva Zacarias. EPM-Unifesp - Departamento de Diagnóstico por Imagem. Rua Napoleão de Barros, 800, Vila Clementino. São Paulo, SP, Brazil, 04023-062. Email: marina_sz@hotmail.com.

a
https://orcid.org/0000-0001-8553-974X

b
https://orcid.org/0000-0002-6556-7003

c
https://orcid.org/0000-0003-0838-3731

d
https://orcid.org/0000-0002-8601-4774

e
https://orcid.org/0000-0002-2019-7918

f
https://orcid.org/0000-0002-2701-1928.

SCIMAGO INSTITUTIONS RANKINGS

Figures | Tables

Figures (10)
Tables (1)

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Figure 1
Axial T2-weighted MRI sequence (A) and axial T1-weighted MRI sequence in late hepatobiliary phase (B), showing dilatation of the intrahepatic bile duct (thick arrows) and sparse cysts (open arrows), some with a central dot of hypointense signal on T2 (thin white arrow), filled with the contrast agent in the late phase (black arrows), proving that there is communication with the bile duct, which is consistent with the diagnosis of Caroli disease.

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Figure 2
Numerous liver cysts and signs of portal hypertension with perigastric collaterals (arrow). A magnetic resonance elastography test (at 5.9 kPa) revealed fibrosis. Such findings are suggestive of congenital hepatic fibrosis.

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Figure 3
Patient with cystic fibrosis. Image shows atrophy and fatty replacement of the pancreas (arrowheads) with gross calcifications in the cephalic region (arrow).

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Figure 4
Periportal halo with a hypointense signal (arrows) in a patient with primary biliary cholangitis.

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Figure 5
A 38-year-old male patient with ulcerative colitis and recurrent cholangitis. A: MRCP image showing stenosis of the common bile duct (open arrow) accompanied by a small area of irregular dilatation of the intrahepatic bile ducts and areas of stenosis (thin continuous arrow) alternated with dilated segments (dashed arrow), creating a beaded appearance; biliary diverticula (arrowhead); and obliteration of the peripheral bile ducts, creating a “pruned tree” appearance (thick arrow). The imaging findings and the clinical context suggest the diagnosis of primary sclerosing cholangitis. B: Schematic diagram.

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Figure 6
MRCP image (A) showing a long segment of stenosis in the common bile duct (arrow) with upstream dilatation of the biliary tree. B: Unenhanced axial T1-weighted MRI sequence showing pancreatic diffuse decreased signal intensity and loss of clefts definition (asterisks), suggestive of autoimmune pancreatitis. The clinical context and imaging findings suggest IgG4-related cholangiopathy. C: A schematic diagram showing prestenotic dilatation (arrowheads) of the intrahepatic bile ducts.

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Figure 7
A 36-year-old male patient with a history of eosinophilic pneumonia who presented with abdominal pain, jaundice, and peripheral eosinophilia. A: Coronal T2-weighted MRI sequence showing abrupt narrowing of the distal intrapancreatic common bile duct (arrow) and upstream dilatation of the biliary tree. The pathology findings were consistent with eosinophilic cholangitis. B: A schematic diagram showing gallbladder wall thickening (arrowhead).

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Figure 8
Patient presenting with thrombosis of the hepatic artery two years after liver transplantation. Coronal T2-weighted MRI sequences (A and B) showing severe dilatation of the biliary tract (arrowheads), hypointense content within the duct (black arrows), and a biloma (asterisk) contiguous to the dilated bile duct. The clinical context and the imaging findings suggest ischemic cholangiopathy. C: schematic diagram showing hepatic duct stenosis (thin white arrows) and hepatic artery thrombosis (thick arrow).

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Figure 9
MRCP image of a patient in the postoperative period after cholecystectomy presenting with iatrogenic stenosis of the right hepatic duct (arrow), which has a low insertion into the common bile duct, near the cystic stump (arrowhead).

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Figure 10
Diagnostic algorithm for non-neoplastic cholangiopathies. ADPKD, autosomal dominant polycystic kidney disease; ARPKD, autosomal recessive polycystic kidney disease; RLL, right liver lobe; AMA, antimitochondrial antibody; PBC, primary biliary cholangitis; CA, carbohydrate antigen; GI, gastrointestinal; GB, gallbladder; RF, rheumatoid factor; ANF, antinuclear factor; IgG4-SC, IgG4-related sclerosing cholangitis; IBD, inflammatory bowel disease; PSC, primary sclerosing cholangitis.

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Table 1
Classification of cholangiopathies.

Figure 1 Axial T2-weighted MRI sequence (A) and axial T1-weighted MRI sequence in late hepatobiliary phase (B), showing dilatation of the intrahepatic bile duct (thick arrows) and sparse cysts (open arrows), some with a central dot of hypointense signal on T2 (thin white arrow), filled with the contrast agent in the late phase (black arrows), proving that there is communication with the bile duct, which is consistent with the diagnosis of Caroli disease.

Figure 2 Numerous liver cysts and signs of portal hypertension with perigastric collaterals (arrow). A magnetic resonance elastography test (at 5.9 kPa) revealed fibrosis. Such findings are suggestive of congenital hepatic fibrosis.

Figure 3 Patient with cystic fibrosis. Image shows atrophy and fatty replacement of the pancreas (arrowheads) with gross calcifications in the cephalic region (arrow).

Figure 4 Periportal halo with a hypointense signal (arrows) in a patient with primary biliary cholangitis.

Figure 5 A 38-year-old male patient with ulcerative colitis and recurrent cholangitis. A: MRCP image showing stenosis of the common bile duct (open arrow) accompanied by a small area of irregular dilatation of the intrahepatic bile ducts and areas of stenosis (thin continuous arrow) alternated with dilated segments (dashed arrow), creating a beaded appearance; biliary diverticula (arrowhead); and obliteration of the peripheral bile ducts, creating a “pruned tree” appearance (thick arrow). The imaging findings and the clinical context suggest the diagnosis of primary sclerosing cholangitis. B: Schematic diagram.

Figure 6 MRCP image (A) showing a long segment of stenosis in the common bile duct (arrow) with upstream dilatation of the biliary tree. B: Unenhanced axial T1-weighted MRI sequence showing pancreatic diffuse decreased signal intensity and loss of clefts definition (asterisks), suggestive of autoimmune pancreatitis. The clinical context and imaging findings suggest IgG4-related cholangiopathy. C: A schematic diagram showing prestenotic dilatation (arrowheads) of the intrahepatic bile ducts.

Figure 7 A 36-year-old male patient with a history of eosinophilic pneumonia who presented with abdominal pain, jaundice, and peripheral eosinophilia. A: Coronal T2-weighted MRI sequence showing abrupt narrowing of the distal intrapancreatic common bile duct (arrow) and upstream dilatation of the biliary tree. The pathology findings were consistent with eosinophilic cholangitis. B: A schematic diagram showing gallbladder wall thickening (arrowhead).

Figure 8 Patient presenting with thrombosis of the hepatic artery two years after liver transplantation. Coronal T2-weighted MRI sequences (A and B) showing severe dilatation of the biliary tract (arrowheads), hypointense content within the duct (black arrows), and a biloma (asterisk) contiguous to the dilated bile duct. The clinical context and the imaging findings suggest ischemic cholangiopathy. C: schematic diagram showing hepatic duct stenosis (thin white arrows) and hepatic artery thrombosis (thick arrow).

Figure 9 MRCP image of a patient in the postoperative period after cholecystectomy presenting with iatrogenic stenosis of the right hepatic duct (arrow), which has a low insertion into the common bile duct, near the cystic stump (arrowhead).

Figure 10 Diagnostic algorithm for non-neoplastic cholangiopathies. ADPKD, autosomal dominant polycystic kidney disease; ARPKD, autosomal recessive polycystic kidney disease; RLL, right liver lobe; AMA, antimitochondrial antibody; PBC, primary biliary cholangitis; CA, carbohydrate antigen; GI, gastrointestinal; GB, gallbladder; RF, rheumatoid factor; ANF, antinuclear factor; IgG4-SC, IgG4-related sclerosing cholangitis; IBD, inflammatory bowel disease; PSC, primary sclerosing cholangitis.

Table 1 Classification of cholangiopathies.

Congenital

Alagille’s syndrome

Caroli’s syndrome

Cystic fibrosis

MDR-3 deficiency

Polycystic liver disease (autosomal dominant polycystic liver disease, autosomal dominant polycystic kidney disease, autosomal recessive polycystic kidney disease)

Immune-mediated

Allograft rejection

Graft-versus-host disease

Primary biliary cholangitis

Idiopathic

Biliary atresia

Idiopathic childhood/adulthood ductopenia

IgG4-related cholangiopathy

Primary sclerosing cholangitis

Eosinophilic cholangitis

Sarcoidosis

Infectious

AIDS-related cholangiopathy

Bacterial cholangitis (caused by Escherichia coli, Klebsiella spp., Enterococcus, Enterobacter, Pseudomonas spp., anaerobic microorganisms, etc.)

Parasitic cholangitis (caused by Ascaris lumbricoides, Opisthorchis viverrini, Clonorchis sinensis, Fasciola hepatica, etc.)

Malignant

Cholangiocarcinoma

Other

Drug-induced cholangiopathy (caused by the use of ketamine, 5-fluorouracil, etc.)

Vascular/ischemic cholangiopathy (e.g., post-transplant hepatic artery stenosis and systemic vasculitis)

Source:

⁽

⁶6 Cheung AC, Pisarello MJL, LaRusso NF. Pathobiology of biliary epithelia. Biochim Biophys Acta Mol Basis Dis. 2018;1864(4 Pt B): 1220-31.

⁾

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Non-neoplastic cholangiopathies: an algorithmic approach

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[1] Correspondence: Dra. Marina Silva Zacarias. EPM-Unifesp - Departamento de Diagnóstico por Imagem. Rua Napoleão de Barros, 800, Vila Clementino. São Paulo, SP, Brazil, 04023-062. Email: marina_sz@hotmail.com.