The liver is one of the most important organs in your body. It's like a big factory. It does a lot of important work, like removing toxins from the food we eat, storing energy, and making bile, which helps us digest food. So, keeping this factory working properly is very important for the health of our entire body. But sometimes there are diseases that silently affect the liver, diseases that we don't hear much about. Today we are going to talk about one such disease. That is Primary Biliary Cholangitis, or PBC as we call it for short.
Simply put, what is PBC?
Okay, let's keep this simple. As I mentioned earlier, the liver produces a fluid called bile . This is what helps us digest the fats in the food we eat, absorb certain vitamins, and get rid of unwanted cholesterol and toxins from the body.
Now imagine, inside the liver there is a network of bile ducts, which are like a small tube system. The bile that the liver makes travels along these small tubes and is stored there, helping to digest food when needed. What happens in PBC is that these bile ducts are damaged.
When these ducts are damaged, bile cannot flow properly and starts to accumulate inside the liver. It's like a broken water pipe filling the house with water. The bile that accumulates in this way starts to damage the liver cells. When this damage occurs over time, scar tissue begins to form instead of healthy liver cells. We call this condition cirrhosis . When the liver becomes scarred in this way, its normal functioning is almost completely impaired.
The four stages of PBC disease
PBC does not get worse all at once. It develops gradually, through several stages. There are usually four main stages.
| Stage | What Happens |
|---|---|
| Stage 1 (Portal Stage) | This is the first stage. What happens here is inflammation, which is a swelling, around the bile ducts. This change begins in the portal area of the liver where the blood vessels and bile ducts join. |
| Stage 2 (Periportal Stage) | Now the inflammation has spread a little further and is starting to come out around the bile ducts. It is at this stage that small amounts of scar tissue (fibrosis) begin to form. |
| Stage 3 (Septal Stage) | By now, that scar tissue has spread further, creating connections between parts of the liver. It's like a spiderweb-like network of scars spreading throughout the liver. |
| Stage 4 (Biliary Cirrhosis) | This is the most severe and final stage of the disease. By this time, scar tissue has spread throughout the liver, thickening the liver and forming lumps. This condition is called cirrhosis. At this stage, the liver's function is greatly impaired. |
What really causes PBC?
This is a problem that many people have. In fact, doctors have not yet found the exact cause of PBC. However, the current accepted opinion is that it is an autoimmune disease caused by a combination of genetic and environmental factors .
Okay, what is this autoimmune disease?
Simply put, this is a malfunction in our body's own immune system. Normally, our white blood cells (specifically T-cells) fight off disease-causing germs. But in PBC, these immune cells go rogue and start attacking healthy bile duct cells in the liver. It's like our own army mistakenly attacking our own people.
It is when our own cells continuously attack the bile ducts in this way that the damage I mentioned earlier begins to occur.
It cannot be said that this disease is passed down from generation to generation through genes. That is, there is no rule that just because a mother has it, her daughter will also develop it. However, if someone in the family, especially a close relative (mother, sister, or brother), has PBC, the risk of others developing this disease is slightly higher than the average person. Therefore, it can be assumed that there is a genetic link.
Who is at higher risk of developing PBC?
Although anyone can develop PBC, some people are at a slightly higher risk. Let's take a look at who they are.
| Risk Factor | More Details |
|---|---|
| Gender (Sex) | This is surprising. PBC is more common in women. Women are about 9 to 10 times more likely to develop the disease than men. |
| Age | Most people diagnosed with this disease are between the ages of 30 and 60 , but it can also occur in people younger or older than that. |
| Family History | As I said before, if someone in your family (especially your mother or sister) has PBC, you are at higher risk of developing the disease than the general population. |
| Genetic Background | Worldwide, this disease is most commonly reported among people from Northern Europe (such as Scotland and Scandinavian countries). However, it can occur in any country and ethnic group in the world. |
What are the symptoms then?
This is the most important thing. The scary thing about PBC is that many people don't show any symptoms in the early stages. It's sometimes discovered incidentally when a blood test is done for another condition.
But as the disease progresses, symptoms such as these may appear:
- Fatigue: A major symptom is feeling extremely tired for no apparent reason, even when sleeping.
- Itchy Skin: You may experience unbearable itching all over your body, especially on your palms and soles.
- Dry eyes and mouth: Decreased production of tears in the eyes and saliva in the mouth, causing a feeling of dryness.
- Joint and bone pain.
- Mild pain or discomfort in the upper right side of the abdomen.
As the disease progresses (to the stage of cirrhosis), symptoms such as swelling of the legs, fluid in the abdomen, and yellowing of the eyes and skin (jaundice) may also occur.
When should you see a doctor?
If you, especially if you are a woman between the ages of 30 and 60, are experiencing symptoms like persistent fatigue and itchy skin for no apparent reason, don't just dismiss it as "just getting older" or "too much work." See your doctor right away and talk about it.
The doctor will examine you and, if necessary, order several blood tests. This disease can be easily diagnosed by checking liver enzymes and specific antibodies (AMA - Antimitochondrial Antibodies). So do not ignore the symptoms. The sooner it is diagnosed, the more likely it is to be treated and controlled.
Take-Home Message
- PBC is a chronic disease that damages the small bile ducts in the liver.
- This is not a contagious disease. It is often caused by a fault in our body's own immune system (autoimmune).
- In the early stages, there may be no symptoms at all. The most common initial symptoms are fatigue and itchy skin.
- This disease is most common among women, especially those between the ages of 30 and 60.
- If you have any doubts or symptoms about this, self-diagnose (Wait, that's Thai. Typo in my thought process. Must be careful). Do not engage in self-diagnosis, but definitely see a qualified doctor and seek advice.
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