Primary sclerosing cholangitis (PSC) is a progressive disease causing scarring (fibrosis) and destruction of the bile ducts. It can involve only the larger ducts, only the small ducts in the liver, or both. There is a strong association of PSC with inflammatory bowel disease, especially ulcerative colitis. The cause of PSC is not known, but classically it affects young adult males with ulcerative colitis.

Diagnosis

The most accurate test to diagnose PSC affecting the larger ducts is ERCP (See Endoscopic Retrograde Cholangopancreaticography). However, because this test is invasive and has a risk of complications, another test called a Magnetic Resonance Cholangiopancreaticogram (MRCP) is usually used instead. This is a form of MRI scan, and is done at a radiology practice. If small duct PSC is suspected, a liver biopsy can be performed to diagnose this.

Treatment

PSC should be managed by a gastroenterologist or hepatologist. It does not respond well to treatment, but its progression can be slowed by the use of a drug called urso-deoxycholic acid. Management involves prevention of and watching for complications of the disease, including:

• Liver cirrhosis
• Osteoporosis
• Cholangitis (infection of the biliary ducts)
• Blockage of the bile ducts
• The development of certain forms of cancers e.g. colorectal cancer and cholangiocarcinoma