Liver Disorder Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a disease that causes an inflammation of the bile ducts. The inflammation damages the bile ducts, which causes scarring or fibrosis, hardening, narrowing and possibly blocking of the bile ducts inside and outside the liver. The bile ducts are the only way for bile to drain from the liver, so narrowed ducts cause bile to back up in the liver. This eventually leads to damage and cirrhosis of the liver.

Causes of Primary Sclerosing Cholangitis

The cause of PSC is still unknown, though scientists do have theories. Some possible causes include viral or bacterial infections. However, most researchers suspect is that PSC is the result of an immunologic disorder because a majority of cases occur in association with other disorders, namely ulcerative colitis or HIV. Since ulcerative colitis and HIV both involve the immune system, and because no virus or bacteria has yet been isolated in PSC, medical experts suspect that primary sclerosing cholangitis is an autoimmune disease.

Who is Most Affected?

Primary sclerosing cholangitis often begins sometime between 30 and 50 years of age. The disease seems to affect men more often than women. As mentioned before, primary sclerosing cholangitis seems to be associated with ulcerative colitis and HIV. Over 70 percent of patients with primary sclerosing cholangitis also have ulcerative colitis.

Symptoms of Primary Sclerosing Cholangitis

For many people with primary sclerosing cholangitis, symptoms develop very slowly and become apparent only after many years. When symptoms do present themselves, fatigue, itching all over the body (pruritis), and jaundice (yellowing of the eyes and skin) are fairly common. Other symptoms that may signify primary sclerosing cholangitis include:

  • chills
  • fever
  • tenderness in the abdomen
  • loss of appetite
  • weight loss.

In later stages of the disease, cirrhosis of the liver may set in and, finally, liver failure occurs.

Diagnosing Primary Sclerosing Cholangitis

Diagnosis of PSC usually starts with signs and symptoms of the disease. If no symptoms are present, a diagnosis is more difficult. The possibility of catching the disease in its early stages still exists if the doctor notices abnormal results on blood tests.

If primary sclerosing cholangitis is suspected, an endoscopic retrograde cholangiopancreatography (ERCP) is performed to determine whether the disease is present. Dye is injected into the bile ducts and x-rays are taken to determine whether the bile ducts have narrowed.

A biopsy of the liver may be performed to gauge the progression of the disease and to determine how much damage has been done to the liver.

Treatments for Primary Sclerosing Cholangitis

No cure currently exists for primary sclerosing cholangitis. Apart from transplants of the liver, all treatments for primary sclerosing cholangitis are focused on alleviating symptoms and complications of the disease: if itching is a problem, medications are available to relieve it; if recurring infections plague the patient, antibiotics are prescribed; if nutritional deficiencies are apparent, dietary supplements are recommended to make up for the deficiencies. For severe blockages of the bile ducts, surgery may be necessary.

Liver transplantation is an option only in severe cases of primary sclerosing cholangitis, when liver failure becomes highly possible. New techniques and new medications to prevent rejection of the transplanted organ make this form of treatment highly successful; approximately 87 percent survive one year after the transplant, and 77 percent survive for over three years. But with the high demand for organs, transplantation may not be a viable treatment option for many until years later.

Resources

American Liver Foundation. (2003). What is primary sclerosing cholangitis?

Beers, M. H.,