Primary biliary cholangitis (PBC) is an autoimmune disorder that affects the small bile ducts. It leads to reduced drainage of bile from the liver cells, inflammation of the liver cells and, ultimately, to liver scarring or liver cirrhosis.

Symptoms and causes

Pathogenesis

The pathogenesis of PBC is not yet fully known. We suspect that environmental factors (e.g. bacteria) cause an activation of the immune system in genetically predisposed patients. This activated immune system attacks the tiny bile ducts in the liver.

In Europe, one to two people per 100,000 inhabitants are diagnosed with the disorder every year and about 2 to 40 people per 100,000 inhabitants have the condition. Of PBC patients, 90% are women. The condition mainly affects women over the age of forty. In the past, the diagnosis of PBC was only made when patients presented with decompensated liver cirrhosis (hence, the former name: primary biliary cirrhosis).

In the past, the diagnosis of PBC was only made when patients presented with decompensated liver cirrhosis (hence ,the former name primary biliary cirrhosis). Therefore, the condition is now often diagnosed before cirrhosis occurs. As a result, the term primary biliary cirrhosis was replaced by primary biliary cholangitis in 2015.

Symptoms

PBC can cause fatigue, itching, dry eyes and dry mouth.

Diagnosis and treatment

Diagnosis

Now, PCB is diagnosed as a result of increased alkaline phosphatase during routine blood sampling. If an ultrasound of the liver does not explain the increase in alkaline phosphatases, we look for anti-mitochondrial antibodies in the blood. These are positive in 95% of patients who have PBC and are proof in the blood for this condition. The condition is therefore now often diagnosed before cirrhosis occurs. As a result, the term primary biliary cirrhosis was replaced with primary biliary cholangitis in 2015.

Treatment

Care for patients who have PBC is based on four pillars.

1. The medicinal treatment of PBC with the aim of inhibiting the progression of the condition. The first line treatment is ursodeoxycholic acid (UDCA) at a dose of 13-15 mg/kg/day. There are also new treatments (obeticholic acid, bezafibrate, etcetera).

2. The treatment of PCB symptoms.

3. Screening for osteoporosis (bone decalcification) using bone densitometry: 30% of PBC patients have osteoporosis.

4. Avoidance of other causes of liver damage such as alcohol, obesity, hepatitis A and B viral infection.

Treatment centres and specialisations

Digestive Centre

Latest publication date: 21/01/2021
Supervising author: Dr Vanderstraeten Erik