Primary biliary cirrhosis is a condition where small and medium sized bile ducts within the liver are inflamed, undergo destruction and scarring. Thus, bile produced by the liver cannot reach the intestines. This leads to accumulation of bile in the liver, resulting in liver damage. The condition progresses to cirrhosis and liver failure in the late stages.
Primary biliary cirrhosis (PBC) is seen more commonly in middle – aged females. It probably occurs due to an autoimmune reaction, wherein the body fails to recognize certain tissues as its own, and launches an attack against them by producing proteins called antibodies. A person may be genetically prone to develop PBC. Certain environmental conditions may also trigger the development or worsening of the disease. These conditions include staying in regions with soil contaminated with toxins, use of hormone replacement therapy, certain cosmetics and smoking. Bacterial or viral infections may also trigger the reaction. Patients with PBC may in addition suffer from other autoimmune diseases like thyroid disease and Sicca syndrome.
Patients with primary biliary cirrhosis survive for 7 to 16 years once the diagnosis is made. However, the survival has now improved due to treatment with urso-deoxycholic acid (UDCA) and liver transplantation.
Patients may suffer from symptoms like fatigue, itching, upper abdominal discomfort and jaundice. The liver and spleen may be larger than normal. The patient may show accumulation of fluid in the abdomen and legs. Detection of anti-mitochondrial antibodies (AMA) in the blood is an important test, which helps to confirm the diagnosis of PBC. It is positive in nearly 90% patients with PBC.
Secondary biliary cirrhosis occurs due to prolonged obstruction of bile ducts outside the liver. This obstruction may be due to narrowing of the duct, cancer or stone in the duct causing prolonged inflammation.
Latest Publications and Research on Biliary CirrhosisThe Genetics of Complex Cholestatic Disorders. - Published by PubMed
Aspartate Aminotransferase-to-Platelet Ratio index (APRi) in infants with biliary atresia: Prognostic value at presentation. - Published by PubMed
Primary biliary cirrhosis developing in a patient with Crohn's disease during the course of infliximab treatment: The first case in the literature. - Published by PubMed
[Ischemic cholangiopathy induced by extended burns]. - Published by PubMed
Evaluation of an automated chemiluminescent immunoassay kit for antinuclear antibodies in autoimmune diseases. - Published by PubMed