Primary sclerosing cholangitis (PSC) is a chronic disease, which leads to an inflammation of the bile ducts connecting the liver to the gallbladder and small intestine. The inflammation causes a narrowing of the ducts and eventually blockages, preventing bile from aiding in the digestion of fats and vitamins.
Pent up bile in the liver can lead to the destruction of healthy liver cells. In addition, scar tissue from the ducts can spread to the liver, potentially causing cirrhosis and organ failure.
The disease is most prevalent in adults, with more than 70 percent of the cases diagnosed in men. However, cases have been discovered in children as well. The average age at diagnosis is 40 years.
Researchers have not established any exact causes for PSC. However, it has been linked to inflammatory bowel disease (IBD). Roughly 75 percent of PSC patients suffer from an IBD called ulcerative colitis.
Researchers also believe a person’s risk of developing PSC is raised if it runs in their family.
The disease takes its toll over time, so symptoms are not typically noticeable – especially in the early onset of PSC. Patients may complain about constant fatigue, severe skin itching and jaundice. These symptoms may appear and fade over time and, gradually, become more severe.
If left undiagnosed, PSC may lead to infected bile ducts, which could cause fevers, chills and increased abdominal pain.
Initially, a doctor will conduct a blood test to evaluate the liver enzyme levels in the patient. If warranted, the doctor will use cholangiography – taking images of the bile ducts – to confirm a case of PSC.
Doctors may use the following methods to get an image of the bile duct:
- Endoscopic retrograde cholangiography (ERCP) – where a tube is inserted through the mouth and proceeds down to the intestinal tract. Then a dye in dispersed and an x-ray taken to determine the extent of the blockage
- Percutaneous transhepatic cholangiography – in this method, doctors will use a needle to insert a tiny tube into a duct in the liver. Dye is then injected into the tube and an x-ray is taken
- Magnetic resonance cholangiopancreatography – in this method, which is gaining wider acceptance, doctors use magnetic resonance imaging (MRI) to secure images of the bile duct. This non-invasive procedure uses radio waves to develop images of the organs.
At this time, available treatments focus solely on alleviating pain from symptoms and managing complications. Doctors may prescribe or recommend ointments to relieve itching, antibiotics to treat infections and vitamin supplements. An endoscopic procedure may be used to eliminate blockages, but this will not prevent future blockages from occurring.