Primary sclerosing cholangitis, first described in the mid-1850s, is a complex liver disease that is heterogeneous in its presentation.
Dr John Eaton and colleagues from Minnesota, USA reviewed the pathogenesis of primary sclerosing cholangitis, and investigated future management.
The team report that primary sclerosing cholangitis is characterized by chronic cholestasis associated with chronic inflammation of the biliary epithelium, resulting in multifocal bile duct strictures that can affect the entire biliary tree.
Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy.
|The recognition of specific clinical subtypes could improve classification of the disease |
The team noted that the etiology of primary sclerosing cholangitis is not fully understood, which in part explains the lack of effective medical therapy for this condition.
However, the researchers acknowledge that there is a better understanding of the molecular pathogenesis of primary sclerosing cholangitis.
The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease.
Dr Eaton's team comments, "We review our current understanding of this enigmatic disorder and discuss important topics for future studies."