What is primary biliary cholangitis?
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic disease that slowly destroys the bile ducts in the liver. The bile ducts are small tubes that carry bile, a fluid made by the liver that helps digest fats and remove toxins from the body. In PBC, inflammation damages the bile ducts, causing them to become scarred and blocked. Over time, this can lead to the build-up of bile in the liver, resulting in liver damage and, in severe cases, cirrhosis (scarring of the liver) or liver failure.
PBC is an autoimmune disease, meaning the body’s immune system mistakenly attacks healthy bile duct cells. It primarily affects middle-aged women, though it can occur in men and women of all ages.
Symptoms of primary biliary cholangitis
The early stages of PBC often show no symptoms, and many individuals are diagnosed during routine blood tests for other conditions. As the disease progresses, symptoms may start to appear and worsen over time. Common symptoms of PBC include:
- Fatigue: One of the most common symptoms, fatigue can be persistent and overwhelming.
- Itchy skin (pruritus): Itching, often intense, is another common symptom and can occur without any visible rash.
- Jaundice: Yellowing of the skin and eyes due to the build-up of bile in the liver.
- Dry eyes and mouth: Many individuals with PBC also experience dryness in the eyes and mouth.
- Pain in the upper right abdomen: Discomfort in this area may be linked to liver inflammation.
- Bone and joint pain: As PBC progresses, patients may experience musculoskeletal discomfort.
These symptoms can vary from person to person, and their severity may depend on the stage of the disease.
Causes and Risk Factors
The exact cause of primary biliary cholangitis remains unclear, but it is believed to be triggered by a combination of genetic and environmental factors. Known risk factors for PBC include:
- Gender: PBC is far more common in women, accounting for approximately 90% of cases.
- Age: The condition most often develops in individuals between the ages of 30 and 60.
- Genetics: People with a family history of PBC are more likely to develop the disease, suggesting a genetic predisposition.
- Infections: Some research suggests that bacterial or viral infections may trigger the onset of PBC in genetically predisposed individuals.
- Autoimmune diseases: People with other autoimmune disorders, such as thyroid disease, rheumatoid arthritis, or Sjögren’s syndrome, are more likely to develop PBC.
Complications of primary biliary cholangitis
If left untreated, PBC can lead to several serious complications, including:
- Cirrhosis: Long-term inflammation of the bile ducts can cause scarring in the liver, which can impair its ability to function.
- Liver failure: In advanced stages of PBC, the liver may lose its ability to perform vital functions, requiring a liver transplant.
- Osteoporosis: People with PBC are at increased risk of developing osteoporosis, a condition that weakens bones, making them more prone to fractures.
- Vitamin deficiencies: Since bile helps absorb fat-soluble vitamins (A, D, E, and K), individuals with PBC may develop deficiencies in these vitamins.
- Increased risk of liver cancer: In advanced stages, patients with cirrhosis due to PBC are at a higher risk of developing liver cancer.
Diagnosing primary biliary cholangitis
PBC is usually diagnosed through a combination of blood tests, imaging studies, and sometimes a liver biopsy. The key diagnostic tests include:
- Blood tests: Liver function tests can reveal elevated levels of liver enzymes, particularly alkaline phosphatase (ALP), which is a hallmark of bile duct damage.
- Antimitochondrial antibody (AMA) test: This blood test looks for antibodies that are present in most people with PBC.
- Imaging: An ultrasound or MRI may be used to examine the liver and bile ducts and rule out other conditions.
- Liver biopsy: In some cases, a small sample of liver tissue may be taken to confirm the diagnosis and assess the extent of liver damage.
Treatment for primary biliary cholangitis
Although there is currently no cure for PBC, treatment focuses on managing symptoms, slowing disease progression, and preventing complications. Treatment options include:
- Ursodeoxycholic acid (UDCA): This medication helps improve bile flow and is often the first-line treatment for PBC. It can slow the progression of the disease in many people.
- Obeticholic acid: Used for people who do not respond well to UDCA, this newer medication can help reduce bile build-up and liver inflammation.
- Symptom management: Medications can be prescribed to manage specific symptoms such as itching, dry eyes, or fatigue.
- Liver transplant: In cases of severe liver damage or liver failure, a liver transplant may be necessary.
Why choose Birmingham Liver Clinic?
At Birmingham Liver Clinic, we provide comprehensive and expert care for individuals diagnosed with primary biliary cholangitis. Our liver specialists offer personalised treatment plans tailored to each patient’s unique needs. We use the latest diagnostic tools and treatment strategies to ensure the highest quality of care.
Contact us
If you have been diagnosed with PBC or are experiencing symptoms of liver disease, contact Birmingham Liver Clinic today to schedule a consultation. Our expert team is here to guide you through diagnosis and treatment, ensuring you receive the support you need.
