Primary Biliary Cholangitis

Primary Billiary Cirrhosis, or PBC, is a chronic, or long-term, a disease that very gradually damages and eventually destroys the medium-sized bile ducts within the liver. Bile is a digestive liquid that is made in the liver and, through the bile ducts, travels to the small intestine to help in digestion and absorption of fats and fat-soluble vitamins (Vitamins A, D, E, and K).

In patients with PBC, the bile ducts are damaged by inflammation. This causes bile and other harmful substances to remain in the liver and build up levels in the blood, which may lead to irreversible scarring of liver tissue, called cirrhosis. As cirrhosis progresses, the liver may lose its ability to function.

PBC advances gradually and progression can be slowed with medication. However, it is a chronic illness and in some cases may lead to life-threatening complications, especially after cirrhosis develops. This means that regular medical checkups by a specialist are very important to monitor the condition.

Causes

Recent studies consider PBC an autoimmune disease, a condition where the body turns against its own cells. In PBC, the body develops active inflammation with an unknown trigger, and the inflammation process injures bile duct cells and eventually, over decades of time, may cause cirrhosis.

Although PBC is technically not a heritable disease, meaning a disease caused by a specific gene or genetic defect that is passed from parent to child, there appears to be some family link. PBC is more common among siblings and in families where one member has been affected.

Risk Factors

Women account for about 90 percent of PBC cases. It is most commonly diagnosed in patients between the age of 35 and 60.

Signs and Symptoms

Most people with PBC do not have symptoms, especially in the early stages of the disease. When symptoms do occur, the most common is pruritus, or severe itching of the skin, often in the arms, legs, and back.

Other symptoms may include fatigue, fluid build-up in the ankles and abdomen, and darkening of the skin. A collection of fatty deposits in the skin around the eye (Xanthelasmas) or over joints and tendons (Xanthomas) can also be seen. Jaundice, or yellowing of the skin and whites of the eye, can also be present in patients with the advanced disease. Several other disorders often occur with PBC. The most common is problems with tear and salivary glands, causing dry eyes or mouth (called Sjogren’s syndrome). Arthritis and thyroid problems may also be present. Renal (kidney) stones and gallstones may develop. Bone softening and fragility (osteoporosis), which causes bones to fracture more easily, can occur in late stages of the disease if vitamin and calcium needs are not being met.

If I’ve been diagnosed with PBC, what questions should I ask my doctor about PBC?

  • How severe is the liver damage?
  • What treatment do you recommend? Will this slow down the progression of the disease?
  • Will any medication be prescribed? What are the side effects?
  • Should I change my diet?
  • Are there any supplements you would suggest that I take? (or avoid!)
  • What can be done to relieve my symptoms?
  • If cirrhosis develops, is transplantation my only option?

Screenings and Diagnostic Tests

Often, patients are diagnosed with PBC after a routine blood test screening result shows elevated Alkaline Phosphatase, one of the several enzyme chemicals present in blood that help us judge if liver inflammation or damage is present. Doctors need to do several tests to confirm a diagnosis of PBC. One test looks for the presence of anti-mitochondrial antibodies (AMA) in the blood. This test is positive in nearly all PBC patients, and seldom in other conditions. An Ultrasound exam is usually done and sometimes other kinds of scans (CAT or MR) may be needed to support the diagnosis. A Liver biopsy, in which a small sample of liver tissue is removed for testing, may be done to further confirm the diagnosis. It is very important to distinguish PBC from a more serious disease called Autoimmune Chronic Hepatitis. About 10% of PBC cases have features of this condition and these diseases overlap. Since treatment and outlook differ considerably, a reliable diagnosis is very important.

Treatment

Patients most often take a daily dose of a drug known as Ursodiol. This drug is a naturally occurring bile acid, which improves the liver’s ability to function in PBC patients and may delay the need for a liver transplant.

The recommended dose is 15 mg per kilogram of body weight (about 300 mg two to three times daily for an average weight person), and the medication must be taken every day for life, or until a liver transplant. Side effects are limited to none, with no long-term dangers.

Other drugs are effective at relieving the symptoms of PBC, including itching. Proper vitamin intake and calcium are very important to avoid complications caused by the limited absorption of these substances due to low levels of bile in the small intestine.

A new highly expensive medication (obeticholic acid, brand name Ocaliva™) is available and being tested for those individuals who do not improve their blood tests taking ursodiol.

Treatment For Itching

Antihistamines such as diphenhydramine (Benadryl, others) and loratadine (Alavert, Claritin, others) are commonly used to reduce itching from insect bites,dry eyes and dry mouth. On the other hand, antihistamines can help with sleep if itching keeps you awake.

    • Cholestyramine (Questran, Prevalite) is a powder that must be mixed with food or liquids. Though cholestyramine works for most people, the taste is unpleasant.
    • Rifampin (Rifadin, Rimactane, others) is an antibacterial drug, which is taken in pill form. Exactly how rifampin reduces itching is unknown, but it may block the brain’s response to itch-inducing chemicals in your circulation.
    • Opioid antagonists such as naloxone (Bunavail, Evzio) and naltrexone (Vivitrol) may help itching related to liver disease. Like rifampin, these drugs seem to reduce the itching sensation by acting on your brain.

Treatment for dry eyes and mouth:

  • Artificial tears and saliva substitutes are available over-the-counter.
  • Pilocarpine (Isopto Carpine, Salagen) and Cevimeline (Evoxac) are prescription medicines that may be used if over-the-counter medications do not help.

Lifestyle and Home Remedies

Diet Restrictions are seldom needed and no specific diet is known to help PBC. Our main concern is to maintain a healthy diet and avoid other liver conditions related to overweight (fatty liver) or excess alcohol. Sufficient vitamins, particularly A, D, E and K are important since they aren’t absorbed efficiently; but always get guidance from your clinician about the correct amounts of vitamins. Maintaining a healthy lifestyle will help patients feel better and may relieve or prevent some of the secondary symptoms of PBC. Your doctor may recommend:

  • A reduced sodium diet (if there is fluid retention)
  • Calcium and Vitamin D supplements (to maintain appropriate body levels)
  • Periodic screening of bone mineral density by a test called DEXA scan (If osteoporosis is present)—
  • Avoid or reduce the consumption of alcohol
  • Proper skin care For dry skin
  • Artificial tears for dry eyes