Primary Sclerosing Cholangitis

Diagnosis

Most often, PSC is first suspected after an abnormality appears in a routine blood test or health evaluation. Mayo specialists use blood tests, cholangiography (an X-ray test) and liver biopsies to diagnose PSC. Many patients have no symptoms and may remain symptom-free for many years. If symptoms occur, they can include:

• Diarrhea
• Itching
• Fatigue
• Jaundice
• Episodes of fever and chills
• Vomiting blood
• Passing black or very dark stools
• Swollen abdomen or feet
• Abdominal pain
• Weight loss

Stages of PSC

Stage 1 — Fibrosis or scar tissue limited to a few spots called portal areas, little areas of "plumbing" or ductwork in the liver tissue
Stage 2 — Fibrosis begins to appear outside the portal areas. The strands of fibrosis are not yet connected to each other.
Stage 3 — Areas of fibrosis connecting to each other
Stage 4 — Widespread, honeycomblike scarring known as cirrhosis

Diagnostic Tests

Blood tests

Levels of alkaline phosphatase (an enzyme produced in the bile ducts) may be elevated, indicating that the bile ducts may be obstructed and that the liver is not properly producing bile.

Levels of aspartate aminotransferase (AST — enzymes produced in liver cells and bile ducts) may also be elevated, indicating liver cell damage.

Total bilirubin measures the amount of bilirubin in the blood. Bilirubin is released into the blood when red blood cells break down. The liver uses bilirubin to make bile. Elevated levels suggest blockage or obstruction.

Cholangiography

This is an X-ray test where dye is injected into the bile ducts, usually through an endoscope. While the patient is sedated, a thin, flexible tube is inserted through the mouth into the part of the intestine where the bile duct drains into the bowel. Dye is then injected into the bile duct and X-rays are taken. This is called percutaneous transhepatic cholangiography (PTC).

Magnetic resonance cholangiography (MRC) is a new technique to look at the biliary tract in a noninvasive manner.

Liver Biopsy

A liver biopsy (tissue sample) may be done to confirm PSC and to determine the severity and extent of the disease. This can be performed on an outpatient basis.

During a liver biopsy, a small piece of tissue is taken from the liver using a needle guided by ultrasound or a CT scan. A pathologist looks at the tissue sample under a microscope to determine changes or damage in the liver tissue.