Primary biliary cholangitis

"In the Clinic we have all the necessary tools for the non-invasive and invasive diagnosis of primary biliary cholangitis. In the same way it is possible to perform radiological tests to rule out the complications of this disease".


Primary biliary cholangitis, formerly known as primary biliary cirrhosis, is a chronic disease that affects the small-bore bile ducts that are distributed in the liver. These bile ducts have the function of collecting the bile produced in the liver and transporting it to the gallbladder where it is stored while we are fasting. After eating, the gallbladder contracts and releases bile into the digestive tract.

In primary biliary cholangitis there is a slow and progressive destruction of these small bile ducts due to unknown causes, although the presence of autoantibodies in the blood of patients with this disease suggests that autoimmune phenomena exist at the origin of the damage.

The continuous destruction and the attempt to regenerate new biliary ducts, maintained during years, can condition the loss of the normal hepatic architecture until the development of a cirrhosis.

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What are the symptoms of cholangitis?

The symptoms depend on the anatomical location and the severity of the inflammation.

In ulcerative colitis the involvement at the rectal level is the most common symptom being the expulsion of blood through the rectum usually accompanied by increased number of stools.

Abdominal pain can be associated more or less diffuse that improves initially with defecation. There may be fever and weight loss depending on the severity, duration and location of the outbreak.

The most common symptoms are:

  • Expulsion of blood from the rectum.
  • Abdominal pain.
  • Fever.
  • Loss of weight.

Do you have any of these symptoms?

You may have primary cholangitis

How is biliary cholangitis diagnosed?

<p>Laboratorio de extracciones</p>

Primary biliary cholangitis is typically diagnosed by elevated cholestasis rates (GGT and Alkaline Phosphatase) and to a lesser extent transaminases (AST and ALT) and elevated cholesterol levels.

In more advanced stages, elevation of bilirubin, decrease of albumin levels, lengthening of prothrombin time and decrease of platelet count can be observed.

Anti-mitochondrial autoantibodies (AMA) are found in 90% of patients with primary biliary cholangitis. Other autoantibodies, such as antinuclear antibodies (ANA), can also be found in 30% of cases.

A liver biopsy may be necessary for diagnostic confirmation. In a small subgroup of patients primary cholangitis occurs together with autoimmune hepatitis (overlap syndrome). In these patients, laboratory data are not sufficient for diagnosis and a liver biopsy is essential. Performing liver ultrasound and elastography allows the detection of complications such as cirrhosis and liver tumor (hepatocarcinoma).

How is primary biliary cholangitis treated?

Treatment with ursodeoxycholic acid improves the clinical course of this disease in most cases, especially if it has been started early.

Recently, the use of obeticolic acid has been introduced for patients who do not respond favorably to treatment with ursodeoxycholic acid.

In more advanced cases, when the disease has produced complications, such as liver cirrhosis, it may be necessary to resort to liver transplantation.

Where do we treat it?


The Hepatology Unit
of the Clínica Universidad de Navarra

We are pioneers in the application of gene therapy in the treatment of liver tumors and hereditary metabolic diseases, and we have extensive experience in the diagnosis and treatment of viral hepatitis and in the treatment of liver cancer using radioembolization systems with Ytrium-90 microspheres. 

The Clinic is at the forefront in Spain in performing liver transplantation between living people.

Treatments we perform

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Why at the Clinica?

  • Highly specialized team of professionals with more than 25 years of experience.
  • Nursing team specialized in hepatic patients.
  • Important research activity on the molecular mechanisms that cause some of these diseases.