Primary Biliary Cholangitis (PBC)

What is Primary Biliary Cholangitis (PBC)?

Primary Biliary Cholangitis (PBC) is a chronic liver disease that primarily affects the small bile ducts within the liver. Bile ducts play a crucial role in transporting bile, a fluid produced by the liver, to the intestines to aid in digestion. In PBC, the immune system mistakenly targets and damages these bile ducts, leading to inflammation and scarring. Build up of bile within the liver and blood stream causes symptoms such as nausea, jaundice and itching.

 

Who is at risk for PBC?

While the exact cause of PBC is unknown, it is more common in middle-aged women, particularly those between the ages of 30 and 60. It is also more prevalent in individuals with a personal or family history of autoimmune diseases.

 

What are the common symptoms of PBC?

Early stages of PBC might not show noticeable symptoms, but if picked up at a later stage, or as the disease progresses, you might experience:

 

  • Fatigue: Feeling tired even with sufficient rest, which can affect quality of life significantly

  • Itchy Skin (pruritis): Intense itching all over the body, especially at night

  • Dry Eyes and Mouth: Due to decreased tear and saliva production

  • Abdominal Pain: Discomfort in the upper right side of the abdomen

  • Jaundice: Yellowing of the skin and eyes

  • Cholesterol Deposits: Small, yellowish bumps under the skin, found around the eyes, elbows and hands

  • Osteoporosis: Weakening of the bones, leading to increased fracture risk

 

How is PBC diagnosed?

PBC can be diagnosed through a combination of medical history, physical examination, blood tests suggestive of bile duct damage, and imaging studies such as an abdominal ultrasound. One crucial blood test is for elevated levels of an autoimmune antibody marker called anti-mitochondrial antibody (AMA), which are commonly found in PBC. A specialist liver scan called a Fibroscan may be used to determine how much liver damage exists. Invasive tests such as a liver biopsy are rarely required to make a diagnosis.

 

What are the complications of PBC?

If left untreated, PBC can lead to serious complications such as:

 

Cirrhosis: Severe scarring of the liver tissue, which impairs liver function

Portal Hypertension: High blood pressure in the portal vein, which can cause varices (swollen veins) and bleeding

Liver Failure: Severe impairment of liver function, which may necessitate a liver transplant

 

How is PBC treated?

While there is no cure for PBC, several treatments can help manage the disease and slow its progression:

 

Lifestyle Changes: A healthy Mediterranean type-diet low in processed foods, coffee, vitamin D, regular exercise, and avoiding/limiting alcohol can help support liver health and prevent complications.

Ursodeoxycholic Acid (UDCA): A medication that can improve liver function and slow down the damage to bile ducts. This is a very safe and generally well tolerated medication. The dose depends on your weight, and if a good response is seen in your blood tests, it should be continued long-term.

Bezafibrate and Obeticholic Acid: Newer medications have been recently developed for PBC that can offer significant benefit to patients who do not respond to UDCA alone.

 

Is there any treatment for itching in PBC?

Topical treatment- menthol aqueous cream can sooth itching and it is important to keep nails short

Antihistamines- these are often prescribed, are generally safe, and may help itching

Other specialist medications- in PBC, patients often require other medications to help get itching under control. These include UDCA, bezafibrate, colestyramine, naltrezone and rifampicin, all of which be prescribed under Specialist Hepatology supervision. Rarely plasma exchange (an invasive method of filtering blood) can be used.

 

When is a liver transplant necessary?

In severe cases where the liver is significantly damaged, a liver transplant might be the best option. A transplant can improve quality of life and increase survival rates for individuals with advanced PBC.

 

Can PBC be prevented?

Currently, there is no known way to prevent PBC since its exact cause is not understood. However, early diagnosis and continued treatment can help manage the disease and minimize its impact on liver function.

 

Summary

Primary Biliary Cholangitis is a chronic liver disease that primarily affects the small bile ducts, leading to inflammation, scarring, and potential complications if left untreated. Early diagnosis, appropriate medical management, and lifestyle changes can make a significant difference in managing the disease and maintaining liver health. If you suspect any symptoms or have concerns about PBC, it is essential to consult a healthcare professional for proper evaluation and guidance.

 

Useful links:

https://britishlivertrust.org.uk/information-and-support/living-with-a-liver-condition/liver-conditions/primary-biliary-cholangitis/

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