Cholangiopathies

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.