This guide provides information about Primary Biliary Cholangitis (PBC). It aims to help you understand the condition in simple terms.
What is PBC?
Primary Biliary Cholangitis (PBC), previously known as primary biliary cirrhosis, is a long-term (chronic) condition affecting the liver. It is thought to be an autoimmune disease, meaning your body’s immune system mistakenly attacks its own tissues. In PBC, the immune system slowly damages the small tubes (bile ducts) inside your liver.
Bile is a fluid made by the liver that helps digest fats and remove waste products. When the bile ducts are damaged, bile cannot flow properly and builds up in the liver. This build-up, called cholestasis, can cause inflammation and scarring (fibrosis) in the liver over time. If left untreated, this scarring can become severe, leading to cirrhosis, where the liver is permanently damaged and cannot function properly.
What Causes PBC and Who Gets It?
The exact cause of PBC is not fully understood, but it likely involves a combination of factors:
- Genetic predisposition: Having certain genes makes you more likely to develop PBC. If a close family member (parent, sibling, child) has PBC, your risk is higher.
- Environmental triggers: Exposure to certain environmental factors might trigger the condition in people who are genetically susceptible. Potential triggers include infections (like urinary tract infections), smoking, and exposure to certain chemicals.
How Common is PBC?
PBC is considered a relatively uncommon or rare condition.
- Incidence: This refers to the number of new cases diagnosed each year. In Europe, the incidence is estimated to be around 1 to 2 new cases per 100,000 people annually. In the UK, this translates to about 2 to 3 new cases per 100,000 people each year.
- Prevalence: This means the total number of people living with the condition at a specific time. In the UK, about 20,000 people are living with PBC, which is roughly 35 cases per 100,000 people. European studies estimate prevalence between 18 and 27 cases per 100,000 people. Globally, estimates vary widely, from 7 to 940 per million people, depending on the population studied.
Who is Most Affected?
- Age: PBC is most commonly diagnosed in people between the ages of 45 and 65, although it can occur in younger adults (rarely before 15) and older individuals.
- Gender: PBC affects women much more often than men. Estimates suggest the ratio of women to men with PBC is around 9:1 or slightly lower in newer studies (4:1 to 6:1).
- Ethnicity and Geography: PBC occurs worldwide and in all ethnicities. However, it appears to be more common in people of Northern European descent and in North America. Some studies suggest cases might be increasing in Asian populations.
What is the Impact of PBC?
PBC affects individuals differently. Many people (up to 60%) have no symptoms when diagnosed, especially in the early stages, and the condition is found through routine blood tests.
Common symptoms, when they occur, include:
- Fatigue: Feeling extremely tired is a common and often debilitating symptom.
- Pruritus (itching): Persistent itching, often worse at night or in warm conditions, is another frequent symptom.
- Other symptoms: Dry eyes or mouth (associated with Sjogren’s syndrome), discomfort in the upper right abdomen, and sometimes jaundice (yellowing of the skin and eyes) in later stages.
Living with PBC and Life Expectancy
PBC develops slowly, often over many years. With treatment, many people can manage their symptoms and slow the progression of liver damage.
- Complications: If the disease progresses, complications can arise, including:
- Cirrhosis and liver failure.
- Portal hypertension (high blood pressure in the vein leading to the liver).
- Difficulty absorbing fat-soluble vitamins (A, D, E, K), potentially leading to bone problems like osteoporosis.
- Increased risk of developing liver cancer (hepatocellular carcinoma or HCC), particularly if cirrhosis develops.
- Life Expectancy: The outlook for people with PBC has improved significantly with treatment.
- If diagnosed and treated early, especially before symptoms develop, life expectancy can be similar to that of the general population.
- People who respond well to the main treatment (ursodeoxycholic acid) generally have a normal life expectancy.
- Without treatment, life expectancy can be reduced, particularly if symptoms are present at diagnosis. Factors like high bilirubin levels (a substance in bile) indicate more advanced disease and poorer prognosis.
Treatments for PBC
While there is currently no cure for PBC, treatments aim to slow down liver damage and manage symptoms:
- Ursodeoxycholic Acid (UDCA):This is the primary treatment. It is a naturally occurring bile acid that helps improve bile flow and reduce liver inflammation. It can significantly slow disease progression and improve survival.
- Second-line Therapies: For individuals who do not respond adequately to UDCA or cannot tolerate it, other medications may be used, often in combination with UDCA:
- Obeticholic Acid (Ocaliva): Approved in 2016.
- Elafibranor (Iqirvo): A newer PPAR agonist approved in the US and Europe in 2024.
- Seladelpar (Livdelzi): Another PPAR agonist granted accelerated approval in the US in 2024.
- Symptom Management: Medications can help manage itching and fatigue. Dietary changes and supplements may be recommended to address nutritional deficiencies.
- Liver Transplant: For individuals who progress to end-stage liver disease, a liver transplant may be the only option.
Ongoing Research
Research continues to explore new treatments and better understand PBC. Clinical trials are investigating new drugs and therapeutic approaches. Recent focus has been on medications like elafibranor and seladelpar, which target different pathways involved in the disease.
Support Groups
Living with a chronic condition like PBC can be challenging. Support groups offer valuable emotional support and practical information.
- PBC Foundation: This is the main UK-based charity specifically for PBC patients and their families. They provide extensive resources, a helpline, online forums, local support groups, and up-to-date information. You can find them online or contact them for support.
- The American Liver Foundation: Offers educational materials and community support.
Remember:
1. This information is intended for general knowledge and educational purposes only, and does not constitute medical advice.
2. It is essential to consult with a qualified healthcare professional for any health concerns or before making any decisions related to your health or treatment.
