A novel hypothesis on PSC

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Lipids Health Dis. 2007 Jan 25;6(1):3

Primary sclerosing cholangitis - The arteriosclerosis of the bile

duct?

Fickert P, Moustafa T, Trauner M

ABSTRACT: Primary sclerosing cholangitis (PSC) represents a chronic

inflammatory disease of the large bile ducts of unknown aetiology and

characterized by periductal fibrosis and stricture formation, which

ultimately result in biliary cirrhosis and liver failure.

Arteriosclerosis involves the accumulation of altered lipids and

lipoproteins in large arteries; this drives inflammation and fibrosis

and ultimately leads to arterial stenosis and hypoperfusion of

dependent organs and tissues. Knowledge of the causative factors is

crucial if disease mechanisms are to be understood and specific

treatment developed. Based on pathogenetic similarities between PSC

and arteriosclerosis, we hypothesize that PSC represents an

arterioSCLEROSIS of the bile duct initiated by toxic biliary lipids.

This hypothesis is based on common molecular, cellular, and

morphological features providing the framework for a deepened

understanding of their pathogenesis and should stimulate

translational research that could offer some promise in the search

for novel treatment strategies for both diseases. PMID: 17254334