Primary Biliary Cholangitis (PBC)
Primary Biliary Cholangitis (PBC), previously known as primary biliary cirrhosis, is a chronic liver disease that occurs when the bile ducts inside the liver, called intrahepatic bile ducts, are gradually destroyed. Bile, produced in the liver, normally flows through these ducts to the small intestine to aid in the digestion of fat and fat-soluble vitamins (A, D, E, and K). When the ducts are damaged, bile builds up in the liver, causing inflammation and scarring (fibrosis). Over time, this can lead to cirrhosis, where healthy liver tissue is replaced by scar tissue, resulting in impaired liver function and associated complications.
Key Facts about Primary Biliary Cholangitis (PBC) in India:
- PBC primarily affects women, but there has been an increase in diagnoses among men.
- PBC is a chronic disease that damages and eventually destroys the small bile ducts in the liver, leading to inflammation.
- It is estimated that around 65 out of every 100,000 women in the United States have PBC.
- The disease typically becomes apparent during middle age, affecting individuals between the ages of 45 to 65 years.
- Although there is no cure for PBC, there are medications available to manage the disease and slow down the progression of liver damage.
Important Information for Newly Diagnosed Individuals:
What are the signs and symptoms of PBC? PBC often progresses slowly, and many individuals may not experience symptoms, especially in the early stages of the disease. The most common initial symptoms include fatigue and itching of the skin. Other symptoms may include:
- Abdominal pain
- Darkening of the skin
- Small yellow or white bumps under the skin or around the eyes
- Dry mouth and eyes
- Bone, muscle, and joint pain
As the disease progresses, symptoms of cirrhosis may develop, including:
- Yellowing of the skin (jaundice)
- Swelling of the legs and feet (edema)
- Enlarged abdomen due to fluid accumulation (ascites)
- Internal bleeding in the upper stomach and esophagus from enlarged veins (varices)
Thinning of the bones (osteoporosis) leading to fractures is another complication of PBC. Additionally, individuals with cirrhosis have an increased risk of liver cancer (hepatocellular carcinoma).
What causes PBC?
- The exact cause of PBC is unknown. It may be related to immune system dysfunction. Although PBC is not technically a hereditary disease, there is evidence of a familial link. It is more common among siblings and in families where one member has been affected.
How is PBC diagnosed?
- Since many people with PBC may not experience symptoms, the disease is often discovered incidentally through abnormal results in routine liver blood tests. If PBC is suspected, a blood test to check for antimitochondrial antibody (AMA) is conducted. This test is positive in nearly all people with PBC. A liver biopsy, where a small sample of liver tissue is taken, can help confirm the diagnosis. Imaging studies may be used to rule out other diseases or further evaluate patients diagnosed with PBC.
How is PBC treated?
- Treating the disease: While there is no cure for PBC, there are medications available to slow down disease progression and manage symptoms. Ursodiol (brand names Actigall, URSO 250, URSO Forte) is a medication that helps move bile out of the liver and into the small intestine. If used early enough, Ursodiol can improve liver function and may delay or prevent the need for a liver transplant. UDCA is effective in more than 50% of patients, but some individuals may not respond adequately or tolerate the medication.
In certain cases, medications such as obeticholic acid (brand name Ocaliva) in combination with UDCA or alternative therapies like fenofibrate may be prescribed. Immunosuppressive medications like prednisone or azathioprine may be used in individuals with the “overlap syndrome” of PBC and autoimmune hepatitis. Liver transplantation is considered when medical treatment no longer sufficiently controls the disease, and end-stage liver disease is present.
Treating the symptoms
- Intense itching, a common symptom of PBC, can be alleviated with over-the-counter antihistamines like diphenhydramine (Benadryl). Other prescribed agents may also help, such as rifampicin, naltrexone, cholestyramine, and sertraline.
Dry eyes can be relieved
- By using artificial tears (eye drops), and a dry mouth may be improved by sucking on hard candy or chewing gum, which stimulates saliva production. Saliva substitutes and certain medications can also be used.
Preventing complications
- Regular blood tests are conducted to monitor deficiencies in fat-soluble vitamins. As PBC progresses, some individuals may need replacement therapy for vitamins A, D, E, and K that are lost in fatty stools. Calcium and vitamin D are commonly prescribed to reduce the risk of osteoporosis. Screening for varices and liver cancer is often recommended as liver function declines over time.
Lifestyle Changes for Managing PBC
- Maintaining a healthy lifestyle can help individuals with PBC feel better and alleviate or prevent some associated symptoms. Upon diagnosis, doctors may recommend the following:
Remember that PBC progresses slowly over several years, and many individuals can lead normal lives for an extended period without experiencing symptoms, depending on the early diagnosis. While there is no cure, adhering to the prescribed medication regimen and maintaining a healthy lifestyle can help slow disease progression and prolong life without complications.
Who is at risk for PBC? Women are nine times more likely than men to develop PBC, making up around 90% of PBC cases. The disease typically occurs during middle age, with most diagnoses between the ages of 35 to 60 years. There is also a genetic component to developing PBC, as it is more common among siblings and in families where one member has been affected.
Questions to Ask Your Doctor: If you have been diagnosed with PBC, here are some important questions to discuss with your doctor:
- How severe is the liver damage?
- What treatment do you recommend, and will it slow down disease progression?
- Will any medications be prescribed, and what are the potential side effects?
- Should I make any changes to my diet?
- Are there any supplements you recommend?
- How can my symptoms be relieved?
- If cirrhosis develops, is transplantation the only option?