Primary Sclerosing Cholangitis | Diagnosis & Treatment

How is primary sclerosing cholangitis diagnosed?

The first steps in diagnosing PSC are a physical exam and medical history. During the physical exam, the doctor will look for look for signs of liver damage and bile duct infection. The doctor may ask if the child has had symptoms such as fatigue, itchiness, unexplained weight loss or abdominal pain.

Information about the child’s and family’s health history will help the doctor assess the likelihood that a child has PSC. This may include whether any family members have PSC and whether the child has had inflammatory bowel disease or autoimmune disease.

If the results of the medical history and physical exam show that a child could have PSC, the following tests can help determine if the disease is present or not with more certainty:

Blood tests

  • Liver function tests test the blood for signs of bile duct damage.

Imaging tests

  • Endoscopic retrograde cholangiopancreatography: A clinician feeds a tube (endoscope) down the throat to the liver and injects dye into the bile ducts. The dye helps make bile ducts visible on an x-ray, which is taken with a tiny camera at end the endoscope. A clinician then examines the image for signs of blocked ducts. This procedure is typically performed with a sedative to help the child stay calm.
  • Percutaneous transhepatic cholangiography: Another imaging test that uses dye to make bile ducts visible on an x-ray. In this case, dye is injected into the abdomen through the skin of the stomach and the x-ray is taken with a camera on the outside of the body.
  • Magnetic resonance cholangiopancreatography: A diagnostic imaging exam that uses radio waves and a computer to create images of the bile ducts for clinicians to examine.

How is primary sclerosing cholangitis treated?

There is currently no cure for PSC. However, there are ways to treat affected bile ducts and treatments to relieve symptoms.

Treatments for narrow bile ducts

It is possible to open blocked bile ducts using endoscopic retrograde cholangiopancreatography. Instead of taking x-rays of the liver, the doctor passes tiny tools through the endoscope to open blocked ducts so bile can travel more easily through them and not get backed up in the liver.

Treatments to relieve symptoms

Depending on a child’s symptoms, the doctor may prescribe medication to relieve itching or antibiotics to treat a bacterial infection of the bile ducts.

Liver transplant

Many people with PSC eventually need a liver transplant. Doctors recommend transplant only after every other treatment option has been tried. Though the time frame differs for every person, children with PSC typically need a liver transplant 20 to 25 years after the first diagnosis.

Everyday habits to keep the liver healthy

The following habits can help keep children with PSC, and their livers, healthy longer:

  • eating a healthy diet
  • maintaining a healthy weight
  • getting vaccinated for hepatitis A and B
  • minimizing exposure to chemicals
  • taking no more than the recommended dosage of any over-the-counter or prescription medication
  • not drinking alcohol