Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is a chronic autoimmune disease that affects the small bile ducts in the liver. The bile produced by your liver travels through small ducts to the small intestine, where it helps to digest fats and certain vitamins (such as A, D, E, and K). When these ducts become damaged, bile can no longer flow properly, leading to a build-up of bile in the liver. This can cause inflammation and scarring, also known as fibrosis.
The cause of PBC is unknown, but it is believed to be an autoimmune disorder. Women are nine times more likely than men to develop PBC, meaning that women make up about 90% of PBC cases. The disease most often develops during middle age and is usually diagnosed in people between the ages of 35 to 60 years.
There is no cure for PBC, but treatments such as ursodeoxycholic acid, and in advanced cases liver transplant can help to slow the progression of the disease and manage symptoms. Early diagnosis and treatment is important to prevent complications such as cirrhosis and liver failure.
Symptoms
- Fatigue.
- Itching.
- Darkening of the skin.
- Small yellow or white bumps under the skin (xanthomas) or around the eyes (xanthelasmas).
- Dry mouth and eyes.
- Bone, muscle and joint pain.
In later stage one can also develop:
- Jaundice.
- Abdominal pain.
- Enlarged abdomen from fluid accumulation (ascites).
- Internal bleeding in the upper stomach and esophagus from enlarged veins (varices).
Diagnosis & Monitoring
The diagnosis of PBC is based on a combination of clinical, laboratory, and imaging tests.
The following tests may be used to diagnose PBC:
- Blood tests:
- Elevated levels of alkaline phosphatase (ALP) and/or gamma-glutamyl transpeptidase (GGT) can be the initial findings of PBC.
- Elevated levels of anti-mitochondrial antibodies (AMA) can be specific to PBC.
- In later stages elevated levels of bilirubin may be seen.
- Liver biopsy: a liver biopsy is a procedure in which a small sample of liver tissue is removed and examined under a microscope. It can confirm the diagnosis of PBC and also assess the stage of the disease.
Malabsorptive Issues
In primary biliary cholangitis (PBC), the destruction of the small bile ducts in the liver can lead to malabsorption of fats and fat-soluble vitamins such as vitamin A, D, E and K. This occurs because bile, which is produced by the liver and stored in the gallbladder, is essential for the absorption of fats and fat-soluble vitamins. When the bile ducts are damaged, the bile cannot flow into the small intestine, resulting in malabsorption.
Primary biliary cholangitis (PBC) has been associated with an increased risk of osteoporosis and osteopenia, which are conditions characterized by low bone mineral density (BMD) and increased risk of fractures. This is thought to be due to a combination of factors including malabsorption of vitamin D and other nutrients, and chronic inflammation. Symptoms of osteoporosis and osteopenia can include bone pain, fractures, and a loss of height. It is important for people with PBC to have regular bone density screenings to assess their risk of osteoporosis and osteopenia.
Primary biliary cholangitis (PBC) is associated with an increased risk of lipid disorders, including hypercholesterolemia and hypertriglyceridemia. This is thought to be due to a combination of factors, including the malabsorption of fats and fat-soluble vitamins caused by the destruction of the bile ducts in the liver, and chronic inflammation. Hypercholesterolemia is characterized by high levels of cholesterol in the blood, which can increase the risk of heart disease and stroke. Hypertriglyceridemia is characterized by high levels of triglycerides in the blood, which can increase the risk of pancreatitis and other complications.
Treatment
The primary treatment for primary biliary cholangitis (PBC) is ursodeoxycholic acid (UDCA), a medication that helps to slow the progression of the disease and reduce inflammation in the liver. UDCA is believed to work by inhibiting the formation of bile acids, which can cause damage to the liver. The goal of UDCA therapy is to reduce the level of alkaline phosphatase (ALP) which is a marker of the activity of PBC.
UDCA is usually taken orally and is typically prescribed in a dose of 13-15 mg/kg/day. Treatment with UDCA may take several months before any improvement is seen, and it may take up to 2 years for the full benefit to be realized. In addition to UDCA, other recommendations for PBC may include:
- Medications to reduce itching, such as antihistamines.
- It is important for people with PBC to consume a well-balanced diet that is high in protein and carbohydrate, and low in saturated fats and cholesterol.
- Calcium and vitamin supplements (especially Vitamin D and K) to prevent malabsorption-related deficiencies.
- Medications to reduce the risk of osteoporosis such as bisphosphonates.
- Medications to lower cholesterol and triglycerides levels and omega-3 fatty acid supplements.
- Lifestyle changes such as avoiding smoking and excessive alcohol consumption, regular weight-bearing exercise and losing weight if necessary.
- In advanced stages liver transplantation may be considered.
It is important to note that PBC is a chronic disease, and treatment is lifelong. Regular monitoring of liver function tests and other markers of the disease is necessary to assess the effectiveness of treatment and make any adjustments as needed. As the ability of the liver to function decreases over time, complications associated with cirrhosis will need to be addressed and treated, and then screening for varices and liver cancer is recommended.
FAQs
Primary biliary cholangitis (PBC) is a chronic liver disease that results in damage to the bile ducts, which can lead to the buildup of bile in the liver and eventually to cirrhosis.
The exact cause of PBC is not known, but it is believed to be an autoimmune disorder in which the body’s immune system mistakenly attacks the bile ducts.
Symptoms of PBC can vary from person to person, but common symptoms include fatigue, itching, jaundice, and abdominal pain.
PBC is usually diagnosed based on a combination of symptoms, physical examination, blood tests, imaging tests, and a liver biopsy.
Treatment for PBC may include medications to control symptoms as well as complications, and liver transplantation in cases of advanced disease.
There is currently no cure for PBC, but treatment can help to control symptoms and slow the progression of the disease. In some cases, a liver transplant may be necessary to treat advanced PBC.
Long-term complications of PBC can include liver failure, cirrhosis, liver cancer, portal hypertension and bone loss. It is important for people with PBC to be regularly monitored by a specialist to detect and manage any complications that may arise.
PBC is not a hereditary disease, but there is a slight increased risk if there is a family history of the disease.
PBC and PSC are both chronic liver diseases that involve damage to the bile ducts. The main differences between PBC and PSC are the location and distribution of the inflammation and the characteristics of the bile ducts. PBC typically affects the small bile ducts in the liver, while PSC affects the large bile ducts inside and outside the liver. PBC is also associated with antimitochondrial antibodies (AMA) which are not present in PSC.
While a healthy diet and certain supplements may help to support liver function and overall health, there is currently no specific diet or supplement that has been proven to treat PBC. However, adequate intake of certain nutrients such as vitamin D and calcium may be important to prevent bone loss.