21 Primary Sclerosing Cholangitis

Clinical Presentation

A young man presents with jaundice, fever, generalized malaise, and right upper quadrant pain.

Fig. 21.1 (A,B) Axial contrast-enhanced CT images show irregularly dilated intrahepatic bile ducts with no focal mass lesion seen. (C) Endoscopic retrograde cholangiopancreatography shows abnormal arborization of intrahepatic bile ducts associated with multifocal strictures and irregularly ductal dilatation.

Radiologic Findings

Axial contrast-enhanced computed tomography (CT) images show irregularly dilated intrahepatic bile ducts with no focal mass lesion seen. Endoscopic retrograde cholangiopancreatography (ERCP) shows abnormal arborization of the intrahepatic bile ducts associated with multifocal strictures and irregularly ductal dilatation (Fig. 21.1).

Diagnosis

Primary sclerosing cholangitis (PSC)

Differential Diagnosis

Human immunodeficiency virus (HIV) cholangiopathy
Ascending cholangitis
Cholangiocarcinoma
Diffuse intrahepatic metastatic disease

Discussion

Background

Primary sclerosing cholangitis is a chronic liver disease characterized by inflammation, destruction, and fibrosis of the intrahepatic and extrahepatic bile ducts that leads to cirrhosis of the liver. It is most commonly seen in men, and 70% of patients are younger than 45 years.