Back to Chronic Cholestasis

Introduction

The pathogenesis of primary sclerosing cholangitis (PSC) is not yet fully elucidated, but it is thought to involve autoimmunity. PSC is characterized by progressive fibrosis and stricturing of both intra- and extra-hepatic bile ducts. There is a strong association between PSC and ulcerative colitis. PSC typically occurs in young males.

History

Fatigue and pruritus are common presenting symptoms. If patients develop abdominal pain, fever and rigors, bacterial cholangitis should be suspected. A patient with acute cholangitis should be hospitalized immediately.

Physical examination

Physical signs are generally absent until the disease is advanced. The followings may be present:

• Excoriation marks (from scratching)
• Stigmata of chronic liver disease (particularly if cirrhosis is present)
• Jaundice

Investigation

Currently, ERCP is the gold standard in the diagnosis of PSC. The characteristic findings are multifocal stricturing and dilatation of intra- and/or extrahepatic bile ducts ('beading' appearance). MRCP (magnetic resonance cholangiopancreatogram) appears promising as a non-invasive method. There are no serological markers specific for PSC, although pANCA test is frequently abnormal.

In patients with any chronic liver injury, the development of thrombocytopenia, coagulopathy or hypoalbuinemia are suggestive of cirrhosis.