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".
DR. DELIA D'AVOLA
SPECIALIST. HEPATOLOGY UNIT
What is primary biliary cholangitis?
Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic liver disease. It damages the smallest bile ducts, which run through the liver.
These ducts collect the bile produced by the liver and carry it to the gallbladder, where it is stored while we are fasting. When we eat, the gallbladder contracts and releases bile into the digestive tract.
In this disease, the ducts are gradually destroyed, and the exact cause is unknown. Autoantibodies are found in the blood of most patients, suggesting an autoimmune origin: the body's own immune system attacks the bile ducts. Studies suggest that, in people with a genetic predisposition, certain environmental factors, such as infections, smoking, or certain chemicals, may trigger this reaction.
The destruction of the ducts and the liver's attempts to regenerate them continue for years. Over time, these processes can alter the liver's normal structure and eventually lead to cirrhosis.
What are the symptoms of cholangitis?
The symptoms of primary biliary cholangitis (PBC) depend on the degree of inflammation and damage to the small bile ducts in the liver.
As the disease progresses, symptoms related to bilirubin buildup and liver damage may develop. Symptom severity varies widely from patient to patient.
With early diagnosis and available treatments, many patients can manage the disease and prevent its progression for years.
The most common symptoms include:
- Persistent tiredness or fatigue.
- Itchy skin (pruritus), particularly intense at night.
- Dry eyes and mouth.
- Discomfort or pain on the right side of the abdomen.
- Jaundice (yellowing of the skin and eyes) in advanced stages.
- Darkening of the skin.
- Swelling of the abdomen or legs when advanced cirrhosis is present.
Have your blood tests shown abnormal liver results?
Our hepatology specialists can assess whether you have primary biliary cholangitis.
How is biliary cholangitis diagnosed?
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?
The main treatment is ursodeoxycholic acid. It does not cure the disease, but it can slow the progression of liver damage and improve the course of the disease in most cases, especially when started early.
Some patients do not respond well enough to this medication or cannot tolerate it. For these patients, the European Medicines Agency authorized elafibranor in 2024 and seladelpar in 2025. Both reduce blood markers of liver damage, and seladelpar has also been shown to improve itching.
The specialist may also prescribe medications to relieve symptoms such as itching.
If the disease leads to liver failure, liver transplantation is considered.
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.
Conditions we treat
Treatments we perform
- Hepatic Arterial Embolization
- Partial splenic embolization
- Genetic study in hepatology
- Liver Radiofrequency
- Liver Radioembolization
- Liver resection
- Liver transplantation
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.
Our team of professionals
