Korean J Radiol.  2016 Feb;17(1):25-38. 10.3348/kjr.2016.17.1.25.

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

• Seo N
• Kim SY
• Lee SS
• Byun JH
• Kim JH
• Kim HJ
• Lee MG

• KMID: 2351160
• DOI: http://doi.org/10.3348/kjr.2016.17.1.25

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.

Keyword

Bile ducts; Sclerosing cholangitis; CT; MRI; Differential diagnosis

MeSH Terms

Adult
Aged
Aged, 80 and over
Bile Ducts/*pathology
Cholangiography/*methods
Cholangitis/diagnosis/*pathology
Cholangitis, Sclerosing/*diagnosis/pathology
Cholestasis/diagnosis/*pathology
Chronic Disease
Constriction, Pathologic/diagnosis
Diagnosis, Differential
Female
Humans
Immunoglobulin G/immunology
Liver/pathology
Magnetic Resonance Imaging/methods
Male
Middle Aged
Tomography, X-Ray Computed/methods
Immunoglobulin G

Figure

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  Fig. 1 Pathology of primary sclerosing cholangitis. Photomicrograph of liver biopsy specimen (original magnification, × 400; hematoxylin and eosin stain) reveals fibrous portal widening with concentric onion-skin fibrosis around interlobular bile duct and moderate degree of mixed inflammatory cell infiltration (arrows).

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  Fig. 2 Primary sclerosing cholangitis in 36-year-old male. A. Contrast-enhanced axial CT image demonstrates mild multifocal wall thickening in intrahepatic and extrahepatic bile ducts (arrows). B. MR cholangiography shows multiple and short segmental strictures in intra- and extrahepatic bile ducts (arrows), as well as diverticular outpouching (arrowheads).

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  Fig. 3 Chronologic change of PSC in 36-year-old male. A. MR cholangiography image depicts multifocal alternating strictures and dilatation of intrahepatic bile ducts (arrows). B. Follow-up endoscopic cholangiography obtained 5 years after MR cholangiography (A) shows obliterated peripheral bile ducts, resulting in "pruned tree" appearance (arrows). Extrahepatic bile duct is dilated (arrowhead). MR = magnetic resonance, PSC = primary sclerosing cholangitis

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  Fig. 4 Pathology of IgG4-related SC. A. Photomicrograph of liver biopsy specimen (original magnification, × 400; hematoxylin and eosin stain) shows fibrous portal widening with concentric fibrosis and moderate degree of mixed inflammatory cells infiltration (arrows) around interlobular bile duct (arrowhead) (curved arrow, arteriole; asterisk, portal venule). B. There are abundant IgG4-positive plasma cells around bile ducts (arrowheads) (original magnification, × 400; IgG4 staining). IgG4 = immunoglobulin G4, SC = sclerosing cholangitis

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  Fig. 5 IgG4-related SC in 77-year-old male. A. Contrast-enhanced axial CT image shows mildly enhanced wall thickening of common hepatic duct (arrow) with mild dilatation of intrahepatic bile ducts (arrowheads). B. MR cholangiography shows focal stricture of intrapancreatic bile duct (arrow), as well as multifocal stricture in right intrahepatic bile ducts (arrowheads). IgG4 = immunoglobulin G4, MR = magnetic resonance, SC = sclerosing cholangitis

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  Fig. 6 Recurrent pyogenic cholangitis in 63-year-old male. A. Nonenhanced CT image shows radiopaque stones (arrows) in dilated bile ducts in right posterior segment of liver. B. Contrast-enhanced CT image shows dilated central bile ducts (arrows) and rounded liver appearance with marked hypertrophy of caudate lobe (arrowhead). C. MR cholangiography image demonstrates bile duct stones as multiple filling defects (arrows) with bile duct stricture (arrowhead). CT = computed tomography, MR = magnetic resonance

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  Fig. 7 Recurrent pyogenic cholangitis with Clonorchis sinensis infestation in 62-year-old male. A. Contrast-enhanced CT image shows diffuse dilatation of intrahepatic bile ducts, especially in peripheral portion of bile ducts (arrows). B. MR cholangiography shows marked peripheral intrahepatic bile duct dilatation without central bile duct dilatation. Multiple filling defects (arrowheads) noted within dilated bile ducts suggest presence of Clonorchis sinensis worms. CT = computed tomography, MR = magnetic resonance

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  Fig. 8 Cholangiocarcinoma arising from recurrent pyogenic cholangitis in 82-year-old female. A. Dilated left IHD (arrowheads) containing IHD stones is noted on contrast-enhanced CT image. B. Patient underwent follow-up CT scan after 4 years. Axial contrast-enhanced CT scan shows newly developed, soft-tissue lesion (arrow) in left lobe of liver causing bile duct dilatation, as well as narrowing of left portal vein. Lesion was confirmed as cholangiocarcinoma after surgery. CT = computed tomography, IHD = intrahepatic bile duct

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  Fig. 9 Ischemic cholangitis after transcatheter arterial chemoembolization for hepatocellular carcinoma in 74-year-old male. A. Nonenhanced T1-weighted axial MR image detects multiple, hyperintense biliary casts in right intrahepatic bile duct (arrows). B. T2-weighted axial MR image depicts multiple bilomas (arrowheads). MR = magnetic resonance

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  Fig. 10 Ischemic cholangitis after hepatic arterial embolization for postoperative pseudoaneurysm in 50-year-old female. A. At acute stage, contrast-enhanced axial CT image shows bile duct necrosis (arrows) and biloma (arrowhead). B, C. Contrast-enhanced axial CT scan (B) and percutaneous cholangiography (C) obtained 3 months following embolization, demonstrate multifocal bile duct stricture (arrows) and dilatation (arrowheads) that are typical features of chronic ischemic cholangitis. CT = computed tomography

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  Fig. 11 AIDS-related cholangitis with cytomegalovirus (CMV) infection in 43-year-old male. A, B. Contrast-enhanced axial CT images show multifocal stricture and wall thickening in intrahepatic bile ducts (arrows). Papillary stenosis with enhancement (arrowhead) is also seen. C. On endoscopic cholangiography, diffuse stricture is noted in intrahepatic bile ducts (arrow). Common duct also shows dilatation and subtle mucosal irregularity (arrowhead). Endoscopic biopsy confirmed CMV infection. AIDS = acquired immunodeficiency syndrome, CT = computed tomography

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  Fig. 12 Eosionophilic cholangitis in 43-year-old male with hypereosinophilia. A. Contrast-enhanced axial CT image demonstrates ill-defined soft-tissue lesions along periportal space (arrows). B. MR cholangiography shows focal smooth narrowing in left hepatic duct (arrows). Lesion was confirmed as eosinophilic infiltration after liver biopsy. CT = computed tomography, MR = magnetic resonance

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  Fig. 13 Summary of imaging features of sclerosing cholangitis. Red lines along bile ducts indicate sites of frequent involvement according to etiologies of sclerosing cholangitis. Primary sclerosing cholangitis (PSC). Both intra- and extrahepatic bile ducts are usually involved in PSC. Beaded and pruned appearance of bile ducts, and diverticulum-like outpouching are characteristic imaging features of PSC. Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC). Most commonly involved location is intrapancreatic bile duct followed by hilar bile ducts. Circumferential and delayed enhanced wall thickening with visible lumen is suggestive of IgG4-related SC. Findings of autoimmune pancreatitis can frequently be accompanied with IgG4-related SC. Recurrent pyogenic cholangitis (RPC). Most frequently involved segments are left lateral segment and right posterior segment of liver. RPC is characterized by intrahepatic bile duct stones, central bile duct dilatation, and decreased arborization of peripheral ducts. Ischemic cholangitis. Most vulnerable location is middle third of common bile duct. At acute stage, biliary casts and bilomas are common. As disease progresses to chronic stage, focal or diffuse bile duct stricture may develop. AIDS-related cholangitis. Long segmental stricture of extrahepatic bile duct and papillary stenosis are key imaging findings. Eosinophilic cholangitis. Wall thickening in proximal common bile duct and cystic duct is commonly seen. AIDS = acquired immunodeficiency syndrome

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  Fig. 14 Systemic approach of sclerosing cholangitis. This diagram shows approach to differential diagnosis of sclerosing cholangitis based on clinical setting, laboratory findings, as well as typical imaging features. AIDS = acquired immunodeficiency syndrome, EHD = extrahepatic bile duct, Hx = history, IBD = inflammatory bowel disease, IgG4-SC = immunoglobulin G4-related sclerosing cholangitis, IHD = intrahepatic bile duct, PSC = primary sclerosing cholangitis, RPC = recurrent pyogenic cholangitis, TACE = transarterial chemoembolization

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