Primary biliary cirrhosis is a rare chronic disease of the liver. Its course can lead to cirrhosis with severe impairment of liver functions.

Definition of primary biliary cirrhosis

Primary biliary cirrhosis, or primary biliary cholangitis, is a disease of the liver characterized by progressive destruction of the intrahepatic bile ducts. These are small tubes that transport bile from the liver to the gallbladder. As a reminder, bile is a viscous liquid synthesized in the liver, stored and concentrated in the gallbladder and discharged into the small intestine to contribute to digestion.

Primary biliary cirrhosis is a chronic disease of the liver. The progressive destruction of the intrahepatic bile ducts impairs bile flow (cholestasis). Bile can no longer be properly transported to the gallbladder. It accumulates in the liver, and leads to inflammation. As this spreads, fibrous scars (fibrosis) develop throughout the liver. This hepatic fibrosis progressively progresses to cirrhosis (advanced stage of hepatic fibrosis).

As a result of primary biliary cirrhosis as well as other chronic liver diseases, liver cirrhosis is characterized by the gradual replacement of healthy liver tissue with fibrous scar tissue. It leads to a severe impairment of liver functions (metabolism, detoxification, etc.).

Cause of primary biliary cirrhosis

An autoimmune cause is suspected. A dysfunction of the immune system (the body’s defense system) leads it to attack healthy cells. The origin of this immune dysfunction, however, remains unknown.

The autoimmune origin is suspected because primary biliary cirrhosis is often diagnosed in people with autoimmune diseases such as rheumatoid arthritis (inflammation of several joints).

People affected by primary biliary cirrhosis

Primary biliary cirrhosis is a rare disease. The annual number of new cases is estimated at 1 case per 100 inhabitants. It was found that the disease manifested itself most often after 000 years, and preferentially in women (40 cases out of 9).

Diagnosis of primary biliary cirrhosis

Primary biliary cirrhosis can be diagnosed incidentally during a routine examination in people without symptoms. However, in the majority of cases, the diagnosis is made in people with signs of liver damage (see the “Symptoms” section for more details).

The diagnosis of primary biliary cirrhosis is based on various examinations:

• doctor’s assessment of symptoms;
• blood tests;
• medical imaging exams, including ultrasound;
• liver biopsy (removal of a fragment of the liver) in a few cases.

Primary biliary cirrhosis has a progressive course. At the onset of the disease, it is estimated that about half of patients have no symptoms. Primary biliary cirrhosis can then present with different symptoms. 

First symptoms

The first symptoms of primary biliary cirrhosis are usually;

• asthenia (fatigue);
• pruritus (itching);
• dry mouth and eyes.

Associated symptoms

As the disease progresses and worsens, other symptoms may appear:

• discomfort in the upper abdomen;
• hyperpigmentation of the skin;
• accumulation of fat in the form of yellowish protuberances on the skin and / or eyelids;
• increase in the volume of the abdomen due to the accumulation of fluid (ascites);
• swelling of the legs due to fluid build-up (edema)
• jaundice (jaundice).

To date, there is no treatment for primary biliary cirrhosis. Management involves slowing the progression of liver damage, relieving symptoms, and treating potential complications.

Support is based on:

• medicines to relieve itching (eg cholestyramine);
• medicines to reduce liver damage (eg ursodeoxycholic acid);
• vitamin and mineral supplements to correct deficiencies that may arise;
• liver transplant in the advanced stages.

The elimination of alcohol and substances that can be toxic to the liver is also essential in order not to accentuate the lesions.

As the origin of primary biliary cirrhosis is not yet known, no prevention could be established.