Primary Sclerosing Cholangitis (PSC)

What is Primary Sclerosing Cholangitis?

If the ducts that lead from the liver to the duodenum sustain enough damage, or experience repeated bouts of cholangitis, gallstones, or other conditions that affect this tissue, the defense mechanism of the body is to develop scar tissue, which can then hinder the movement of bile secretions. Increased scarring leads to more damage, and forces bile back into the liver, which damages liver cells.

What Causes PSC?

While many conditions are implicated in the development of PSC, no root cause has yet been determined. It is thought that genetics may play a role in PSC, as well as possibly viruses and bacteria. Additionally, it has been noted that many patients with PSC also have immune problems and/or suffer from ulcerative colitis.

What are the symptoms of PSC?

Primary sclerosing cholangitis develops over time, as repeated attacks of gallstones and cholangitis cause the hardening of the duct walls. Although it can affect people of all ages, it primarily affects men over the age of forty.

Symptoms may include:

  • Fatique
  • Jaundice in the skin or eyes
  • Chills and fever

Diagnosis

Blood tests to check levels of liver enzymes are the first step in diagnosing primary sclerosing cholangitis. Doctors confirm the diagnosis using cholangiography, which provides pictures of the bile ducts.

Cholangiography can be performed in the following ways:

  • ERCP — an endoscope (a long, flexible, lighted tube) enters the mouth, slides down the esophagusm, traverses through the stomach, and finally into the duodenum (first part of the small intestine) where dye can be injected (into the bile ducts) and X-rays are taken. ERCP also can be used to take a tissue sample or to treat blocked ducts.
  • Percutaneous transhepatic cholangiography — a needle is inserted through the skin and a thin tube is placed into a duct in the liver. Dye is injected through the tube and x-rays are taken.
  • MRCP/MRI — uses radio waves and magnets to scan internal organs and tissues, including the bile ducts without the use of x-rays or the insertion of instruments into the body.

Other testing may include ultrasound exams and a liver biopsy. Ultrasound uses sound waves to create images of organs inside the body. A biopsy involves removal of a small piece of tissue for examination with a microscope.

Treatment

Although researchers have studied many treatments, none has been shown to cure or slow the progress of primary sclerosing cholangitis. Treatment of PSC aims to relieve symptoms and manage complications. Medical treatment may include various medications to relieve itching, antibiotics to treat infections, and vitamin supplements. Instruments passed through an endoscope during ERCP can help open blocked bile ducts. Liver transplantation may be an option if the liver begins to fail.

Complications

Primary sclerosing cholangitis can lead to various complications, including:

  • deficiencies of vitamins A, D, E, and K
  • infections of the bile ducts
  • cirrhosis (an extensive scarring of the liver)
  • liver failure
  • bile duct cancer

Summary

Primary sclerosing cholangitis inflames, scars, and blocks bile ducts inside and outside the liver. When these bile ducts become blocked, bile builds up in the liver and causes damage to the liver cells. PSC can lead to vitamin deficiencies, infections, bile duct cancer, cirrhosis, liver failure, and the possible need for a liver transplant. The exact cause of PSC is not known, but many people with PSC also have ulcerative colitis, an inflammatory bowel disease. Medications can be used to treat symptoms and complications of PSC.