Primary Biliary Cholangitis: Symptoms and Causes of This Liver Disease


Primary biliary cholangitis (primary BC) is a liver disease that was previously known as primary biliary cirrhosis. It does not affect the liver directly. It affects primarily your bile ducts, which are located in the liver. This disease slowly destroys the bile ducts. Well, what is the implication of this? Bile production occurs in the liver, then, the bile ducts transport it out of the liver. When the disease destroys the bile ducts, bile would no longer be able to leave the liver. The backing up of bile in your liver may then cause liver damage due to irreversible scarring (liver cirrhosis). Liver cirrhosis is a very serious health condition because it can lead to liver failure and death in the long run. In this article, we will talk about primary cirrhosis symptoms and causes. We will also talk about primary biliary cirrhosis treatment.

Bile is very vital for digestion. It is also vital for the removal of toxins and cholesterol, as well as worn-out erythrocytes (red blood cells) from your body. If bile is unable to leave the liver, these functions would be impaired. This could also lead to some fatal health complications. Many health conditions could cause damage to the bile duct. If this is the case, it is called secondary biliary cholangitis. Primary BC, on the other hand, is usually an autoimmune condition. This means that your immune system is attacking and destroying healthy cells in error. This is often due to a genetic predisposition that is triggered by environmental factors.

Primary Biliary Cirrhosis Symptoms

Most people who have primary biliary cholangitis experience no noticeable symptoms whatsoever. The first indication of the disease is usually its diagnosis.

Most people do not visit the hospital for any symptoms related to primary BC. They may be visiting for another reason entirely when their blood tests would show that they have the condition.

It usually takes a long time for symptoms of primary BC to eventually develop. Symptoms do not usually show up earlier than five years with the condition. It can even take up to 20 years for some people to start experiencing symptoms.

It is always better if you find out that you have primary BC in its early stage before you start having symptoms. By the time symptoms show up, the disease is already advanced and is more difficult to manage.

If you do not have symptoms yet at diagnosis, you would have better outcomes. The prognosis is quite poor of you already have symptoms before the diagnosis.

But then, if the condition is advanced, common symptoms that you may have are as follows:

  1. Fatigue
  1. Dryness in your mouth and eyes
  1. Itchy skin

These three symptoms are usually the first to show up. At this point, the disease is already entering its advanced stage. If treatment does not begin to slow down the damage, the following symptoms would show up:

  • Pain around the right upper part of your belly
  • Swelling of your spleen
  • Muscle, joint, and/or bone pain, as well as other musculoskeletal problems
  • Swollen ankles and feet due to an accumulation of fluid (edema)
  • Ascites
  • Xanthomas – Fatty deposits on the area of skin that surrounds your eyes, as well as on your eyelids. Fat may also build up in the creases of your soles, palms, soles, knees or elbows.
  • Jaundice – Yellowing of your eyes and skin
  • Hyperpigmentation (Darkened skin)
  • Osteoporosis (weak bones)
  • High cholesterol levels
  • Diarrhea and/or steatorrhea
  • Underactive thyroid

Primary Biliary Cholangitis Causes

This is where issues come up when we talk about primary BC. Experts cannot clearly tell us what causes this disease. At the end of the day, most experts think it is an autoimmune condition.

Your immune system is responsible for preventing harmful organisms from invading your body and destroying your health. So when there is a signal that a harmful organism is in your body, your immune system moves swiftly before it destroys your health.

So then, your immune system must be able to recognize foreign organisms different from your body cells and tissues. There is bound to be a problem when your immune system can no longer tell the difference. This is what happens in autoimmune diseases like primary BC.

In autoimmune disease conditions, your immune system begins to see your normal body cells as harmful organisms. Then, it begins to destroy them in error, thinking it is protecting your body from harm.

When there is primary BC, the accumulation of some white cells in your liver would cause inflammation. These white cells are components of your immune system. They are called the T cells (or T lymphocytes).

The T cells which usually detect and destroy germs like bacteria. But in the case of primary BC, these T cells begin to mistakenly destroy your bile ducts.

They usually attach the epithelial cells that line your bile ducts. This leads to inflammation, which then spreads to the liver. This eventually leads to cell death and liver damage.

Your body tries to heal up the liver and develops scar tissue to replace the dead cells. However, scar tissues cannot perform the function of healthy tissues. This impairment of function due to scarring is called cirrhosis.

Risk factors for primary BC include the following:

  1. Gender – Women have primary BC more often than men
  1. Age – The condition is most common between the ages of 30 and 60.
  1. Genetic factors, which are evident in a family history of primary BC.
  1. Environmental factors such as bacterial, parasitic, or fungal infections, as well as toxic chemicals and smoking.
  1. People in North America and northern Europe have more people with primary BC.

Primary Biliary Cholangitis Treatment

Autoimmune issues have no specific cure. What you can do is to slow down its progression and prevent complications. You should work together with your doctor to achieve this. Some helpful tips are as follows:

  1. Monitor and treat portal hypertension with your doctor’s help
  1. Do regular exercise to strengthen your weak bones by increasing bone density. You could also take vitamin D supplements to treat osteoporosis.
  1. Vitamin deficiencies are common in people with primary BC. Bile may not be available for the absorption of fat-soluble vitamins. So you need to supplement vitamin A, vitamin D, vitamin E, and vitamin K.
  1. Avoid nutritional supplements and herbs except you get the express consent of your doctor.

Primary biliary cirrhosis is an autoimmune condition. However, primary biliary cirrhosis symptoms do not show up until the disease is in its advanced stage. Primary biliary cirrhosis is often treated by slowing down its progression, managing existing complications, and preventing further complications.

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