Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center.

PURPOSE: To compare the cross-sectional imaging findings of immunoglobulin G4-related sclerosing cholangiopathy (IgG4-SC) and cholangiocarcinoma (CCA).

METHODS: Retrospective search of radiology and pathology databases identified 24 patients with IgG4-SC and over 500 patients with CCA from January 2009 to December 2016. Patients with no pre-treatment imaging studies available on PACS, non-contrasted imaging only, presence of mass lesions, metastatic disease or biliary stents were excluded. 17 patients with IgG4-SC and a selected group of 20 (age and gender matched) patients with CCA were obtained. Images were blinded and independently reviewed by two radiologists. Differences in proportions and means between groups were analyzed using Fishers and Mann-Whitney tests, respectively.

RESULTS: Both readers identified a statistically significant difference in the presence of abrupt common bile duct narrowing between IgG4-SC and CCA (6.7% vs. 68.4%, p < 0.001; 33.3% vs. 75%, p = 0.019). No difference was seen in biliary wall thickening, wall enhancement, extrahepatic exclusive location of disease, or pancreatic duct dilation. Inter-observer variability was κ = 0.52. Total bilirubin and CA 19-9 were unable to differentiate between IgG4-SC and CCA. Serum IgG4 was positive in two of six IgG4-SC patients who were tested.

CONCLUSION: IgG4-SC and CCA share many clinical and imaging findings on CT and MRI. Abrupt bile duct cut sign strongly favors CCA. In the absence of this finding, IgG4-SC should be considered in the differential diagnosis in all cases of suspected extrahepatic CCA.