Definition

Primary biliary cholangitis, previously known as primary biliary cirrhosis, is a disease that occurs due to damage to the bile ducts in the liver. The bile ducts transport bile from the liver to the small intestine, and they all converge into a passage known as the biliary tract. Bile plays a crucial role in the digestion process by aiding in the breakdown of fats and facilitating the absorption of fat-soluble vitamins such as A, D, E, and K in the small intestine.

Cholangitis is an inflammation of the bile duct, primarily occurring in the liver. Several known types of cholangitis include primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), secondary cholangitis, and immune-related cholangitis.

When the bile ducts swell and become inflamed, the condition impedes the flow of bile into the small intestine. This alteration leads to the formation of scar tissue in the liver, known as biliary cirrhosis. If left untreated, liver cirrhosis can progress to liver organ failure.

Primary biliary cholangitis has four clinical stages, depending on the extent of impact and damage to the liver:

• Stage 1: Inflammation and damage to the moderately sized bile duct walls are present.
• Stage 2: Obstruction in the small bile ducts.
• Stage 3: Scar tissue is found in the liver organ and bile ducts.
• Stage 4: Cirrhosis begins to appear, and there is permanent and severe damage or scar tissue formation in the liver organ, resulting in severe organ damage.

Primary biliary cholangitis commonly occurs in middle-aged women worldwide, with the ratio of this disease in women to men being approximately 9:1. The diagnosis is typically determined in women between the ages of 30 and 60 years.

Causes

Primary biliary cholangitis is an autoimmune disease in which the immune system attacks healthy cells instead of harmful foreign substances. In this case, the immune system targets liver cells and damages the bile duct. The exact cause is still unknown, but it is believed to be induced by a combination of genetic factors in the patient and environmental factors acting as triggers.

Because primary biliary cholangitis is an autoimmune disease, this condition can be related to other autoimmune diseases such as:

• Celiac disease, an autoimmune disease that attacks the small intestine.
• Raynaud's disease, a rare blood vessel disorder affecting fingers and toes.
• Thyroid gland disorders.

Risk factor

Some risk factors that can increase the risk of developing primary biliary cholangitis, such as:

• Family history of primary biliary cholangitis
• Having other concurrent autoimmune diseases
• Being female and aged between 30 and 60 years
• Urinary tract infections
• Smoking
• Previous exposure to toxic chemicals
• Undergoing hormone replacement therapy

Symptoms

The symptoms of primary biliary cholangitis develop slowly over time. You may not have symptoms for years, and the disease may be discovered in routine check-ups. The most common symptoms include easy fatigue, dry mouth, dry eyes, and itchy skin. Primary biliary cholangitis can also progressively damage the liver.

Other symptoms may include:

• Abdominal pain
• Dark spots on the skin
• Nausea
• Loss of appetite
• Weight loss
• Xanthoma or yellow lumps around the eyelids
• Joint, muscle, and bone pain
• Jaundice or yellowish skin and eyes
• Swelling abdomen due to fluid accumulation
• Edema or swelling of the ankles and soles of the feet
• Diarrhea

Diagnosis

The diagnosis of primary biliary cholangitis is determined through a medical interview, physical examination, and additional diagnostic tests. Sometimes, the disease is found during additional diagnostic tests in symptomless patients. During the medical interview, the doctor inquires about the symptoms experienced, and it is crucial to provide detailed information about when the symptoms started. This information is vital for establishing a specific diagnosis. 

The doctor will proceed to a physical examination to find specific signs that may lead to liver or bile duct disease. In primary biliary cholangitis, the doctor may observe the accumulation of fluid that causes swelling around the abdominal area or feet. They can also find yellowing of the eyes and skin.

Additional diagnostic tests play a crucial role in determining the diagnosis of primary biliary cholangitis. The diagnostic tests that can be performed include:

• Blood tests, including liver enzyme examinations and liver function test
• AMA (Antimitochondrial Antibody) to indicate autoimmune disease
• Liver biopsy by taking a small sample of liver tissue  for further examination.
• Imaging of the bile duct using ultrasonography, CT scan, MRI, and X-ray imaging.

Management

Primary biliary cholangitis may not exhibit symptoms for around 10 years. If the patient is in the early stage of Primary biliary cholangitis, the life expectancy is generally good. However, if it reaches a stage where the symptoms are severe, such as in the advanced stage, the life expectancy is around 10-15 years. It's important to note that individual experiences can vary, and some people may live longer than others with the same disease.

Currently, there is no specific cure for primary biliary cholangitis. As this disease is progressive, continuous, and becomes more severe, the treatment aims to improve clinical symptoms and protect the liver from further damage. 

If itching is present, a cold compress can be applied to reduce the itch temporarily in the affected area, and wearing baggy or loose clothes with soft material may help. The doctor may also prescribe Ursodeoxycholic acid with proper instructions. In some cases, liver transplantation may be considered if the damage to the liver is too severe.

Complications

The complications of primary biliary cholangitis result from the accumulation of bile that should flow into the small intestine but is retained in the liver, affecting nearby organs such as the spleen and gallbladder. Some complications include:

• Enlargement of the spleen
• Formation of gallbladder stones
• Elevated levels of blood cholesterol
• Osteoporosis and increased risk of bone fractures
• Vitamin deficiencies
• Cirrhosis
• Liver organ failure

Prevention

Currently, there are no specific preventive measures to avoid primary biliary cholangitis. However, individuals can adopt certain lifestyle practices to potentially reduce the risk, such as maintaining a balanced and nutritious diet, avoiding smoking, limiting alcohol consumption, engaging in regular exercise, and ensuring adequate rest.

When to see a doctor?

If your current symptoms worsen, it is advisable to consult with an internal medicine doctor. The doctor will conduct a medical interview and physical examination and may recommend additional diagnostic tests to determine the underlying condition or disease and prescribe the appropriate and necessary treatment.

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