Understanding Primary Biliary Cholangitis (PBC)

Primary biliary cholangitis (PBC) is a chronic, progressive liver disease that causes inflammation of the liver. PBC involves the progressive destruction of bile ducts in the liver, called the intrahepatic bile ducts. When the liver produces bile, it travels along the intrahepatic bile ducts to the small intestine, where it facilitates the digestion of fats and fat-soluble vitamins. When the bile ducts are destroyed, this leads to the buildup of bile, which eventually causes liver inflammation and scarring, and may lead to other damage to the liver such as cirrhosis of the liver and portal hypertension. Currently, the best protection against developing severe liver damage due to primary biliary cholangitis is to understand the symptoms, treatment options, and lifestyle changes necessary for managing PBC.

What Are Symptoms of Primary Biliary Cholangitis?

The symptoms of primary biliary cholangitis often develop slowly and may vary among individuals. Common symptoms include:

• Fatigue: A significant and debilitating tiredness is one of the first and often the most pronounced symptoms of PBC.
• Itching (Pruritus): Also one of the earliest symptoms, it can affect any part of the body and is usually worse at night.
• Dry Eyes and Mouth: Many people with PBC experience dryness, often due to associated conditions like Sjögren’s syndrome.
• Jaundice: Yellowing of the skin and eyes may occur with PBC, indicating liver dysfunction.
• Abdominal Pain: Discomfort or pain may be present in the upper right side of the abdomen.
• Hyperpigmentation: Darkening of the skin, especially in areas exposed to the sun, may occur.
• Muscle and Joint Pain: PBC patients may experience generalized aches and pains in their muscles, bones, and joints.
• Xanthomas: Fatty deposits under the skin, usually around the eyes, may occur with PBC as a result of liver failure.

Can Primary Biliary Cholangitis Be Cured?

Unfortunately, there is currently no cure for primary biliary cholangitis. However, treatments can help manage symptoms, slow disease progression, and improve quality of life. The most common medications used to treat primary biliary cholangitis and associated symptoms include:

• Ursodeoxycholic Acid (UDCA): UDCA is a bile acid that can help move bile through the liver more effectively and is the standard treatment for PBC. If used early, it can improve liver function and help prevent or delay the need for a liver transplant. 
• Obeticholic Acid (OCA): For those who do not respond well to UDCA, OCA can be used to further slow the progression of the disease. OCA increases the flow of bile from the liver and also suppresses the production of bile in the liver. This reduces the exposure of the liver to toxic levels of bile acids. 
• Alternative Therapies: In patients who are incomplete responders to UDCA, other therapies may be used, including fenofibrate. Medications to suppress the immune system may also be prescribed, which may include prednisone or azathioprine.
• Symptomatic Treatments: Medications are commonly prescribed to manage symptoms associated with PBC, including itching, fatigue, and dry eyes.

In advanced cases, when the liver is significantly damaged, a liver transplant may be necessary.

What Are the Stages of Primary Biliary Cirrhosis?

Primary biliary cholangitis progresses through several stages, which can be identified through liver biopsy:

1. Stage 1 (Inflammatory Stage): Inflammation of the bile ducts.
2. Stage 2 (Ductular Proliferation and Periportal Fibrosis): Proliferation of bile ducts and beginning fibrosis around the portal areas.
3. Stage 3 (Septal Fibrosis): Significant fibrosis extending to the liver septa.
4. Stage 4 (Cirrhosis): Extensive scarring and cirrhosis, leading to liver failure.

What Foods Should You Avoid if You Have PBC?

Diet can play a crucial role in managing PBC. Eating a balanced diet rich in fruits, vegetables, lean proteins, and whole grains can significantly support liver health if you have PBC. Foods to avoid include high fat foods such as fatty meats, fried foods, and high-fat dairy products that can exacerbate liver problems. Alcohol should be completely avoided to reduce additional liver stress. Processed foods are high in sugars and preservatives and should be minimized or removed altogether. Avoiding salt is also essential, as high salt intake can lead to fluid retention and worsen liver health.

What Can Trigger PBC?

The exact cause of PBC is not known, but several factors can trigger the disease or may exacerbate its symptoms. PBC is thought to be an autoimmune disease, and family history of PBC or other autoimmune diseases increases one’s risk of developing PBC. Environmental factors are also suspected of contributing to the development of PBC. Exposure to certain harmful chemicals and toxins may trigger the disease. Infections may also be a trigger of PBC. Some bacterial and viral infections are thought to potentially cause the immune response leading to PBC.

What Part of the Body Itches With PBC?

The itching associated with PBC, known as pruritus, can affect any part of the body, though it is often most intense on the arms, legs, and back. The itching can be severe and is typically worse at night, affecting sleep and quality of life.

What Age Does Primary Biliary Cholangitis Start?

PBC can start at any age but it is most commonly diagnosed in middle-aged women, typically between the ages of 40 and 60. It is much less common in men and younger individuals of any gender.

When to Talk to Your Doctor

It is important to speak with your doctor if you experience symptoms of PBC or if you have risk factors such as a family history of the disease. Early diagnosis and treatment can help manage symptoms and slow the progression of the disease. You should contact your healthcare provider if you notice any of the following:

• Persistent fatigue that doesn’t improve with rest
• Unexplained itching, especially at night or if it disrupts sleep
• Yellowing of the skin or eyes
• Abdominal pain or swelling
• Unusual dryness of the eyes and mouth

Your doctor may perform blood tests to check for antimitochondrial antibodies (AMA), a common marker of PBC, and liver function tests. Imaging studies and liver biopsy may also be used to confirm the diagnosis and assess the stage of the disease.

Primary biliary cholangitis is a challenging disease that requires ongoing management and care. While there is no cure, medications like ursodeoxycholic acid and obeticholic acid can slow the disease’s progression and improve quality of life for people with primary biliary cholangitis. Maintaining a healthy diet, avoiding triggers, and seeking early medical intervention are essential for managing PBC. If you experience symptoms or have a family history of the disease, we invite you to consult with one of our healthcare providers at Allied Digestive Health today for proper diagnosis and treatment. Contact us today to make an appointment!

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