Primary Biliary Cirrhosis: Definition, Causes, Symptoms, Diagnosis, and Treatment

Primary biliary cirrhosis definition

Primary biliary cirrhosis is a condition when the bile ducts are slowly damaged. Bile is a fluid produced by the liver that plays a role in the digestion of fat and the production of metabolic waste substances.

This disease is classified as an autoimmune disorder and is now referred to as primary biliary cholangitis (biliary cholangitis). This condition occurs because immune cells mistakenly attack the bile system.

When the bile ducts are damaged, bile can flow back to the liver and cause permanent wound tissue in the liver.

Over time, more and more wound tissue is formed, causing hardening of the liver known as cirrhosis.

What causes primary biliary cirrhosis?

PBC occurs when white blood cells that serve to protect the body from germs actually damage healthy cells that line the gallbladder in the liver. This damage will increase widely and spread to the cells and tissues of the liver.

Damaged and dead cells and tissues will be replaced with connective tissue, which eventually leads to cirrhosis of the liver.

It is not yet known what causes PBC. However, there are several factors that are known to increase a person’s risk of developing PBC, namely:

• Aged 30-60 years
• Female gender
• Have a family suffering from PBC

In addition, a combination of genetic and environmental factors, such as infections, smoking habits and exposure to cigarette smoke, as well as exposure to certain chemicals, can also trigger PBC.

Symptoms of primary biliary cirrhosis

Most people with primary biliary cholangitis do not experience symptoms that are clearly visible. This condition is often detected accidentally when blood tests are performed for other health conditions. Symptoms will develop over the next 5 to 20 years.

Symptoms of primary biliary cholangitis in the early stages

• Always feeling tired
• Itchy skin caused by the buildup of toxins in the body
• The eyes and mouth become dry.

Symptoms of primary biliary cholangitis at an advanced stage

• Pain in joints, bones or muscles
• Upper right abdomen hurts
• Weight loss
• Swelling of the feet and ankles (edema)
• The skin and eyes become yellowish (jaundice)
• Swelling of the spleen
• Accumulation of fluid in the abdominal cavity due to liver failure (ascites)
• Fatty deposits (xanthelasma) on the skin around the eyes, eyelids or folds from the palms of the hands, soles of the feet, elbows and knees
• Skin that becomes darker (hyperpigmentation) that is not caused by sun exposure
• Osteoporosis that can cause fractures
• High cholesterol levels
• Diarrhea with steatorrhea (oily stool)

Primary biliary cirrhosis diagnosis

Your doctor diagnoses biliary cholangitis based on your and family’s medical history, physical exam, and medical exam results. First of all, your doctor will ask about your symptoms and whether you:

• Have a history of autoimmune disorders,
• Have family members who have been diagnosed with bile duct disease, as well as
• Have been exposed to infection or exposed to certain chemicals.

After that, the doctor will perform an examination on your stomach.

Doctors generally check the sound of the stomach with a stethoscope, pressing on certain areas of the abdomen to look for the source of pain, as well as confirming whether your liver and spleen are enlarged.

When needed, your doctor may also suggest follow-up examinations such as the following.

• Blood test. This test can describe the condition of liver enzymes such as SGOT and SGPT, the presence or absence of anti mitochondrial antibodies, and the patient’s blood cholesterol.
• Imaging tests. This test includes X-rays, ultrasounds, and CT scans.
• Liver biopsy. The doctor takes a sample of liver tissue for further examination in the laboratory.

Primary biliary cirrhosis treatment

PBC treatment aims to treat symptoms, slow the further liver damage, and prevent complications. To relieve symptoms, the doctor will give the following medicines:

• Antihistamines, to relieve itching
• Artificial tears, to cope with dryness of the eyes.

As for slowing liver damage, your doctor may give you a number of medications, such as:

• Obeticholic acid, to help improve liver function.
• Ursodeoxycholate, to help the removal of bile fluid from the liver, so that it can improve liver function and reduce scarring in the liver.
• Methotrexate and colchicine, to suppress the immune system.

If the above medications are no longer able to control the development of PBC, and if the patient begins to experience liver failure, then the doctor may recommend a liver transplant.