Let's be aware of Primary Biliary Cholangitis (PBC), a silent disease that affects the liver

Do you often feel tired or itchy for no reason? Sometimes we may not pay much attention to these things as normal things in our daily lives. However, these can sometimes be a warning sign from within our body. In particular, they can be the first signs of a problem with our liver. Today we are going to talk about a disease that can silently affect the body. That is Primary Biliary Cholangitis, or PBC for short.

Simply put, what is Primary Biliary Cholangitis (PBC)?

Imagine that inside your liver there is a system of very fine, tiny tubes. It's like the branches of a tree branching out. These are what we call bile ducts. The main function of these ducts is to carry the fluid called 'Bile' produced by the liver out of the liver, that is, into our digestive system. This bile is very important for us, because it is this bile that helps dissolve the fats in the food we eat.

Now, in a disease called Primary Biliary Cholangitis (PBC), our own immune system mistakenly attacks these bile ducts. This causes inflammation, or swelling, in the bile ducts. Over time, this inflammation damages the bile ducts and gradually destroys them.

What happens then? Like a clogged water pipe, bile can't flow and gets stuck inside the liver. The stuck bile starts to damage liver cells. As this damage continues, scar tissue starts to form in place of healthy liver tissue. Eventually, the liver gradually becomes hard and its function decreases. We call this condition cirrhosis . In the past, this disease was also called Primary Biliary Cirrhosis.

Is this disease really serious?

PBC is a progressive and chronic disease. This means that it does not get better overnight, and symptoms may worsen over time. In the early stages, there may be no symptoms at all. However, if the disease is not controlled, it can eventually lead to liver failure. At that point, it can be life-threatening if a liver transplant is not performed.

Who is most likely to get this disease?

One thing that stands out when talking about this disease is that it is very common among women . For every man who gets this disease, about 10 women get it. There is a huge gap.

• The disease is often diagnosed after the age of 40.
• Also, if someone in your family has other autoimmune diseases , you may be at a higher risk of developing one, suggesting that there may be a genetic link.

What are the symptoms and complications of PBC?

Many people don't experience any symptoms in the early stages of the disease. It's sometimes discovered incidentally when a blood test is done for another reason. But as the disease progresses, symptoms begin to appear.

Common symptoms that come first

• Fatigue: This is the most common symptom. No matter how much sleep or rest you get, the fatigue you feel doesn't go away.
• Itchy skin: Itching can occur, especially on the palms of the hands and soles of the feet. However, it can occur anywhere on the body. For some people, the itching can be unbearable.

These symptoms do not occur in the same way for everyone. For some, they appear right at the beginning of the disease, while for others, they appear much later.

Complications that may occur as the disease progresses

When bile cannot flow properly, it starts to accumulate in our blood. Also, problems arise because the digestive system does not receive bile. As the liver scars increase, pressure also builds up on the blood vessels that pass through the liver. This can lead to various complications. Let's look at these in a table.

What is the real cause of PBC?

As we mentioned earlier, PBC is an autoimmune disease . Simply put, our body's "army" (the immune system) mistakenly attacks our own good cells. It's like attacking our own countrymen, thinking they're enemies. In PBC, this "attack" occurs on the bile ducts in the liver.

Doctors still don't know exactly why this happens, but they believe there are several possible causes:

1. Genetic factor: If someone in the family has these diseases, others are more likely to develop them as well.

2. Environmental factors: When a virus, bacteria, or chemical enters the body, the immune system of a genetically predisposed person may be triggered to function incorrectly.

How is this disease diagnosed? (Diagnosis)

When you tell your doctor about your symptoms, he or she will examine you and order several tests to confirm the diagnosis.

1. Blood Tests: This is the most important test. There are two things that are specifically looked at.

• Antibodies: About 95% of people with PBC have a specific antibody called Antimitochondrial Antibody (AMA) in their blood.
• Liver Enzymes: When the liver is damaged, certain enzymes (especially Alkaline Phosphatase - ALP ) increase in the blood.

2. Imaging Tests: If blood tests suggest PBC, you may be referred for a scan to look at the liver and gallbladder. This can also rule out other causes of your symptoms. An ultrasound scan is usually the first test done. You may also be referred for an MRI scan to get clearer images.

3. Liver Biopsy: Sometimes (especially in people who do not have AMA antibodies in their blood), a very small piece of liver tissue is taken and examined under a microscope to confirm the diagnosis and determine the exact extent of liver damage.

What are the treatments for PBC?

There is no cure for PBC yet. However, there are very effective treatments to reduce the damage to the liver caused by the disease, control the progression of the disease, and manage symptoms.

Medication

• Ursodeoxycholic acid (UDCA): This is the main medication for PBC. It is a naturally occurring bile acid. This medication helps remove bile from the liver and reduces liver damage. It can be very effective when started early in the disease.
• Other medications: For those who do not respond well to UDCA, your doctor may recommend other medications, such as obeticholic acid . In addition, newer medications, such as seladelpar and elafibrinor, are now being used.
• Medications for symptoms:
• For itchy skin: Medications like Cholestyramine, antihistamines, and ultraviolet light therapy are used.
• For vitamin deficiencies: Supplements containing vitamins A, D, E, and K are given.
• For fatigue: Some people may find some relief from stimulant medications like modafinil.

Surgery

If your liver function continues to decline and is not controlled with medication, your doctor may put you on a waiting list for a liver transplant . Liver transplants for PBC are very successful. You can live a normal life after the transplant. Although it is possible for PBC to return to the new liver, it is very rare.

What should I know about living with this disease?

PBC often progresses very slowly. It can take 15-20 years for the disease to reach its final stages. If the disease is diagnosed and treated early, severe cases of the disease can be prevented or delayed. Many people manage their symptoms with medication and live for years without major disruption to their daily lives.

However, some people may have a more rapid progression of the disease. Excessive fatigue and elevated bilirubin levels in the blood may be signs that the disease is progressing rapidly.

Things you can do to protect your liver

Following a good lifestyle and eating habits will help keep your liver healthy for as long as possible.

• Completely stop smoking, alcohol, and unnecessary medication. These are very harmful to the liver.
• Eat more whole foods such as fresh vegetables, fruits, and herbs instead of processed, packaged foods.
• Reduce saturated fats and choose foods that contain unsaturated or "good" fats (e.g. avocados, nuts, olive oil).
• Get some exercise every day. Even something as simple as walking is good. Weight-bearing exercise helps keep bones strong.

Living with a chronic illness like this can be mentally exhausting. Talk to your doctor about ways to reduce your physical, mental, and emotional stress. Seek counseling if needed. Remember, your overall health is much bigger than this illness.

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