Cholangiopathies

Jeong-Min Lee¹

¹Radiology, Seoul National University Hospital, Seoul, Korea, Republic of

Synopsis

Cholangiopathies refer to chronic diseases that involve cholangiocytes, which are the epithelial cells that line the bile ducts. These cells have an important role in the modification of bile volume and composition, are activated by interactions with endogenous and exogenous stimuli (eg, microorganisms, drugs), and participate in liver injury and repair. Cholangiopathies include entities such as primary biliary cholangitis (previously known as primary biliary cirrhosis), primary sclerosing cholangitis, IgG4-associated cholangitis, drug-induced cholangiopathy, secondary sclerosing cholangitis (ischemic, infectious (AIDS)…), cystic fibrosis, ductal plate malformations and idiopathic adult ductopenia. Initial history taking, clinical examination and laboratory findings can reveal important elements for differential diagnosis. Cholangiopathies account for substantial morbidity and mortality given their progressive nature, and the difficulties associated with clinical management. Furthermore, they usually result in end-stage liver disease requiring liver transplant to extend survival. However, differential diagnosis is important, because specific management exists and prognosis can be different according to the type of disease. Furthermore, differentiating benign conditions from malignancy (cholangiocarcinoma) is important, since their treatment and prognosis vary. However, it still remains a big challenge for radiologists and clinicians.

Cholangiopathies refer to chronic diseases that involve cholangiocytes, which are the epithelial cells that line the bile ducts. The definitions of cholangiopathy range from broad to quite narrow. These cells have an important role in the modification of bile volume and composition, are activated by interactions with endogenous and exogenous stimuli (eg, microorganisms, drugs), and participate in liver injury and repair. Cholangiopathies include entities such as primary biliary cholangitis (previously known as primary biliary cirrhosis), primary sclerosing cholangitis, IgG4-associated cholangitis, drug-induced cholangiopathy, secondary sclerosing cholangitis (ischemic, infectious (AIDS)…), cystic fibrosis, ductal plate malformations and idiopathic adult ductopenia. Initial history taking, clinical examination and laboratory findings can reveal important elements for differential diagnosis. Cholangiopathies account for substantial morbidity and mortality given their progressive nature, and the difficulties associated with clinical management. Furthermore, they usually result in end-stage liver disease requiring liver transplant to extend survival. However, differential diagnosis is important, because specific management exists and prognosis can be different according to the type of disease. Furthermore, differentiating benign conditions from malignancy (cholangiocarcinoma) is important, since their treatment and prognosis vary. However, it still remains a big challenge for radiologists and clinicians. Imaging procedures as well as tissue sampling can complete the work-up. Among the noninvasive imaging techniques such as US, CT, and MR imaging, contrast material–enhanced MR imaging with MR cholangiopancreatography plays an important role in the evaluation of patients with suspected cholangiopathies, while offering the most comprehensive evaluation. Although endoscopic retrograde cholangiopancreatography with tissue biopsy or surgery is needed for the definitive diagnosis of many cholangiopathies, certain MR imaging–MR cholangiopancreatographic features of the abnormally narrowed biliary segment may help make a diagnosis. Nevertheless, this can be a difficult procedure and a multidisciplinary approach is indispensable for management.

Acknowledgements

No acknowledgement found.

References

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Proc. Intl. Soc. Mag. Reson. Med. 25 (2017)