What is Primary Biliary Cholangitis?

Understanding Primary Biliary Cholangitis

Primary Biliary Cholangitis (PBC) is a chronic autoimmune disease that affects the bile ducts in the liver. The immune system mistakenly attacks the small bile ducts, causing inflammation and gradual destruction. Over time, bile builds up in the liver, leading to scarring (fibrosis) and eventually, cirrhosis or liver failure if left untreated. The condition is progressive, but treatments like Livdelzi are available and can help slow its development and manage symptoms.

Treatment Options for Primary Biliary Cholangitis

Treating PBC focuses on slowing liver damage, managing symptoms and preventing complications. Medication is the first line of treatment, and new therapies have significantly improved outcomes.

Livdelzi (elafibranor)

Livdelzi is the most recently approved treatment for PBC. It works by activating specific receptors in the liver that regulate bile acid production, helping to reduce inflammation and improve liver function. Livdelzi is typically used when other first-line treatments are not effective or cannot be tolerated. Studies have shown that it helps lower key liver enzyme levels and slows disease progression.

Ursodiol (ursodeoxycholic acid)

Ursodiol is a bile acid medication and one of the standard treatments for PBC. It helps improve bile flow and reduces liver enzyme levels. For many patients, Ursodiol slows the disease, and in some cases it may halt progression altogether. It is taken daily and is most effective when started early in the disease.

Obeticholic Acid (Ocaliva)

Ocaliva is often prescribed for patients who do not respond well to Ursodiol. It works by targeting specific receptors involved in bile acid regulation. While it can be effective in lowering liver enzymes and improving liver function, it may cause itching and should be used under close medical supervision.

Fibrates

Fibrates, such as bezafibrate or fenofibrate, are used off-label in some cases of PBC. They are primarily used to manage high cholesterol but have shown promise in improving bile acid levels and reducing inflammation in PBC patients, particularly when used alongside Ursodiol.

Liver Transplant

In advanced cases where the liver is severely damaged, a liver transplant may be necessary. Although this is a last-resort option, liver transplants for PBC generally have good long-term outcomes.

Managing Symptoms and Lifestyle Changes

In addition to medications, many people with PBC benefit from lifestyle changes and symptom management strategies.

Itching

One of the most common symptoms of PBC is itching, which can range from mild to severe. Treatments include antihistamines, cholestyramine (a bile acid binder) and newer drugs like rifampin or naltrexone. Keeping skin moisturized and avoiding hot showers can also help reduce itching.

Fatigue

Chronic fatigue is another common challenge. While there's no single treatment, strategies like regular light exercise, pacing daily activities and ensuring adequate sleep can be helpful. In some cases, medications like modafinil may be prescribed to improve energy levels.

Dry Eyes and Mouth

PBC is often associated with Sjögren’s syndrome, an autoimmune condition that causes dryness. Over-the-counter eye drops, saliva substitutes and staying hydrated can relieve symptoms.

Bone Health

PBC patients are at higher risk for osteoporosis due to impaired vitamin absorption. Bone density tests, calcium and vitamin D supplements and weight-bearing exercise are important preventive measures.

Diet and Nutrition

Maintaining a healthy, well-balanced diet is key. Since fat absorption may be affected, patients may need fat-soluble vitamin supplements (A, D, E and K). Avoiding alcohol and maintaining a healthy weight can also reduce liver strain.

Common Symptoms of Primary Biliary Cholangitis

Recognizing the early signs of PBC can lead to earlier treatment and better outcomes. Common symptoms include:

• Fatigue.
• Itchy skin (especially at night).
• Dry eyes and mouth.
• Abdominal discomfort in the upper right side.
• Dark urine.
• Yellowing of the skin or eyes (jaundice).
• High cholesterol levels.
• Bone or joint pain.

Some people with PBC may not experience noticeable symptoms for years, and the disease may be found during routine blood tests.