What is Primary Biliary Cirrhosis

Primary Biliary Cirrhosis affects the bile ducts in the liver. It is characterised by chronic inflammation and progressive destruction and fibrosis of the ducts lead to an obstruction to bile flow from the liver and cirrhosis.

Statistics on Primary Biliary Cirrhosis

The disease is significantly more common in women – 95% of patients are women. The peak age at presentation is 30-65 years. However, the disease has been recorded in women as young as 22, and as old as 93.

The incidence of primary biliary cirrhosis is increasing, but the reason is unclear.

Risk Factors for Primary Biliary Cirrhosis

The cause is unknown (possibly autoimmune).

There is a hereditary component, since it is much more common in first-degree relatives.

Progression of Primary Biliary Cirrhosis

The condition is progressive, although it might be initially asymptomatic. However, the rate of progression varies between individuals. In majority of patients without symptoms, in 2-4 years time the symptoms will develop; while one-third of the patients without symptoms remain the same after many years. Hence there is no reliable predictor concerning the onset of symptoms.

Primary biliary cirrhosis will eventually lead to liver failure if liver transplantation is not offered. Liver failure results in symptoms (jaundice, itching, easy bruising, tummy distension).

How is Primary Biliary Cirrhosis Diagnosed?

  • Blood Tests– would have abnormal liver function tests.
  • Coagulation profile – clotting function can be affected with liver dysfunction later in disease.
  • Also high serum cholesterol and high serum immmunoglobulins (IgM) are seen. Immunoglobulins are antibodies in the body.

Prognosis of Primary Biliary Cirrhosis

It may take anywhere between 5 to more than 20 years from diagnosis to progress to fulminant liver failure. Patients who initially present with pruritus have a better prognosis than patients who present with jaundice. Liver transplantation allows for more prolonged survival.

Patients with prolonged advanced disease are at a higher risk of developing liver cancer.

How is Primary Biliary Cirrhosis Treated?

Treatments for PBC are largely depending on the patient’s symptoms.

Itching can be a very frustrating and disabling symptom – treatments of benefit include ursodeoxycholate, cholestyramine, and other newer treatments such as rifampicin and naloxone hydrochloride.

Nutritional supplements such as fat soluble vitamins (A,D,K) and calcium should be given because malabsorption is common with primary biliary cirrhosis. Sometimes additional therapies such as restriction on dietary fat and pancreatic enzyme supplements may be needed.

Sometimes primary biliary cirrhosis is associated with thyroid disease, especially hypothyroidism. If this is the case the patient will need thyroid hormone replacement.

Xanthomas (see above) occur in less than 10% of the patients with primary biliary cirrhosis. Sometimes this may need plasmapheresis, the the blood cholesterol is removed like how the blood is cleansed in dialysis in kidney disease patients.

Other symptomatic treatments will be directed towards lack of red blood cells and fatigue.

As for the treatment for the underlying process itself, the only approved therapy is using ursodeoxycholic acid (UDCA). Other medications such as methotrexate and colchicine are used for many years in the treatment but their values remain unproven.

A liver transplantation should be considered for patients with serum bilirubin or intractable pruritis. However, recurrence of the original disease in the graft does occur.

Primary Biliary Cirrhosis References

  1. Braunwald, Fauci, Kasper, Hauser, Longo, Jameson. Harrison’s Principles of Internal Medicine. 15th Edition. McGraw-Hill. 2001.
  2. Kumar P, Clark M. Clinical Medicine. WB Saunders 2002.
  3. Up to Date: Clinical manifestations, diagnosis, and natural history of primary biliary cirrhosis [online]. 2005. [Cited 2005 September 29th]. Available from: URL: http://www.utdol.com/application/topic.asp?file=hep_dis/8639&type=A&selectedTitle=1~46
  4. Up to Date: Overview of the treatment of primary biliary cirrhosis [online]. 2005. [Cited 2005 September 29th]. Available from: URL: http://www.utdol.com/application/topic.asp?file=hep_dis/9088