Credit: Adobe Stock/Anna

For patients living with primary biliary cholangitis (PBC), understanding the disease and its management is crucial for maintaining liver health. The Educated Patient spoke with Areej Mazhar, DO, a physician at Carolinas Medical Center in North Carolina, to shed light on PBC, its symptoms, causes and treatment options. Mazhar offers expert guidance on recognizing early signs, managing daily symptoms including fatigue and itching, and the latest available therapies.

In addition to medical treatment, lifestyle choices — including nutrition — play an essential role in supporting liver health. Mazhar shares a delicious and liver-friendly turkey and vegetable stir-fry recipe, originally featured as part of the American Liver Foundation (ALF)’s 30-day meal plan. This simple, nutrient-packed dish is a great option for those looking to make healthy eating choices while managing PBC or other liver conditions.

Turkey and Vegetable Stir-Fry

Ingredients:

• 8 ounces ground turkey (98% lean)
• 2 cups mixed vegetables (such as bell peppers, broccoli and snap peas; can use fresh or frozen)
• 2 tablespoons low-sodium soy sauce
• 1 cup brown rice

Instructions:

1. In a small pot, bring water to a boil. Add brown rice and cook until soft (about 40 minutes).
2. In medium saucepan, cook ground turkey in a pan until browned. Add mixed vegetables to pan and stir until tender-crisp. Stir in low-sodium soy sauce and cook for two minutes.
3. Allow to sit for about five minutes, then serve over cooked brown rice.

What is PBC, and how does it affect the liver?

Areej Mazhar, DO: PBC is an autoimmune liver disease that leads to inflammation and destruction of the bile ducts. Both environmental and genetic factors are thought to play a role in the development of PBC.

What are the early signs and symptoms of PBC that patients should watch for?

AM: Patients with PBC can be asymptomatic. Those who have a family history of PBC or other autoimmune diseases (especially CREST syndrome, Raynaud’s or scleroderma) should be cognizant of an association with PBC. The signs and symptoms of PBC can include fatigue, itching in the palms and bottom of the feet specifically or all over the body. Right upper abdominal pain can also occur but is less common. Additionally, fatty deposits on the eyelids called xanthelasmas have often been seen in patients with PBC, though the pathophysiology is unclear.

What causes PBC, and is it considered an autoimmune disease?

AM: PBC is considered an autoimmune liver disease caused by self-antibodies that are against components in the mitochondrial membranes of biliary epithelial cells. Antibodies associated with PBC include antimitochondrial antibodies (AMA), sp-100 and gp210.

What treatment options are available for PBC, and how do they work?

AM: Treatment options can include treatment for the symptoms of itchiness as well as ursodiol (UDCA) and obeticholic acid (OCA). UDCA is a weight-based medication given at a dose of 13 to 15 milligrams per kilogram of body weight. UDCA has anti-inflammatory, cell protective effects and increases the amount of bile produced/released by the liver. Treatment response is monitored by following serum liver tests. OCA is a farsenoid X receptor agonist. It also has the effect of increasing the amount of bile produced/released by the liver as well as affects the synthesis, secretion, absorption and metabolism of bile acids. OCA is usually a secondary treatment offered when there has not been a target decrease in the alkaline phosphatase value to less than 1.67 times the upper limit of normal after a year of UDCA use. OCA can be used with or without UDCA; however, it is contraindicated in patients with decompensated liver disease or cirrhosis.

How can patients with PBC manage their symptoms, such as fatigue or itching, on a daily basis?

AM: Fatigue is a difficult symptom to treat and there is no specific medication to treat this. At times, fatigue can be associated with low vitamin D levels, which can be replaced through oral supplementation. This is not unusual as PBC affects fat-soluble vitamin absorption and vitamin D is one of these fat-soluble vitamins. Regarding itching, lifestyle modifications like moisturizing the skin, avoiding hot baths, avoiding tight clothing and using ice packs can be considered. Medical treatment for itching can include antihistamines, opiate antagonists like naltrexone, rifampicin, selective serotonin reuptake inhibitors (SSRIs) like sertraline, and cholestyramine.

What advice do you have for newly diagnosed patients who may feel overwhelmed?

AM: I would encourage newly diagnosed patients who may feel overwhelmed to join online societies where they can interact with others with the same diagnosis so that they do not feel alone and feel supported through a shared experience. There are several PBC societies including the PBCers, PBC Foundation, and Canadian PBC Society. Additionally, I would recommend patients to feel comfortable discussing any questions or concerns regarding PBC with their physician. Physicians may also recommend counseling to help with emotional and psychological support and well-being.

Is there anything else you would like to add?

AM: I would also recommend female first-degree relatives at age 30 of patients with PBC (women in the family who share half of their genetic information with another person) to consider having a screening test with their primary care provider where an alkaline phosphatase value can be checked. If elevated, AMA can be checked. Additionally, patients with PBC should also be cognizant of fat-soluble vitamin deficiencies, levels of which can be checked and replete, as well as osteoporosis and hypothyroidism.

In addition to liver-healthy recipes, the ALF offers a simple liver health quiz, available in English and Spanish. To learn more about liver health, including education, awareness and screening, visit Think Liver Think Life, and to help researchers find better treatments and cures for liver diseases, visit ALF’s Patient Registry.