Oh my, do you also feel like you are very tired, tired even without doing anything? Or do you sometimes feel like you are scratching your skin for no reason? Sometimes, although we don't pay much attention to these, they can be a sign of a problem with the liver, one of the most important organs in our body. Today we are going to talk about such a disease that affects the liver, and although the name may seem a little complicated, it is easy to understand. That is (Primary Biliary Cholangitis) , also known as (PBC) for short.
What is Primary Biliary Cholangitis (PBC)? Let's understand it simply!
Okay, this name seems a bit long, doesn't it? Let's see what it means.
"Cholangitis" is an inflammation of your bile ducts . You know, the fluid called bile, which is produced in our liver, travels through a system of small tubes. Those tubes are called bile ducts. "Biliary" also means something related to these bile ducts. "Primary" means "basic" or "initial."
Then, when you put all this together, Primary Biliary Cholangitis means that, not due to any other disease, infection, or blockage, but because of this disease, there is an inflammation of the bile ducts inside your liver . Since this inflammation does not occur in response to anything, you don't know when it will stop. So it is a condition that progresses gradually.
This disease was previously called Primary Biliary Cirrhosis because it can eventually cause the liver to become hardened and scarred, leading to a condition called cirrhosis .
How does (PBC) affect our body?
Imagine, what happens if a part of our body is constantly inflamed? Usually, temporary inflammation is part of the healing process. But if this kind of chronic inflammation continues, instead of healing, the area starts to scar.
That's what happens to the bile ducts inside your liver in PBC. These ducts become narrowed and blocked by scarring . Then the bile produced by the liver can't flow properly. This bile starts to back up inside the liver. When this bile builds up, it damages the liver cells and causes inflammation in the liver. Over time, this healthy liver tissue is replaced by scar tissue. Eventually, the liver becomes unable to function properly, which is what we've mentioned before (cirrhosis) .
Is PBC a serious condition?
Yes, Primary Biliary Cholangitis is a chronic and progressive disease . This means that it doesn't just get better, it can get worse over time. It doesn't get worse all at once, it gets worse slowly, through different stages. You may not have any symptoms at first.
However, the most dangerous thing about this is that it can eventually lead to complete liver failure and even death if a liver transplant is not performed.
But, fortunately, there are medications that can slow the progression of this disease . And not everyone goes to this final stage. So don't worry.
What is the difference between (PBC) and (PSC)?
You may have heard of another condition called Primary Sclerosing Cholangitis, or PSC. The two are very similar, but there are a few key differences.
- In PBC , only the bile ducts inside your liver (intrahepatic bile ducts) are affected.
- However, in PSC, the bile ducts inside the liver as well as outside the liver (extrahepatic ducts) are affected .
- There is also some difference in the populations in which these two diseases are most common.
- The important thing is that, although there are medications to treat (PBC), no successful treatment has yet been found for (PSC) .
Who is most likely to develop this disease (PBC)?
Although this may seem surprising, women are about ten times more likely to develop PBC than men (10:1) . According to statistics in the United States, about 60 out of 100,000 women have the disease, compared to about 15 out of 100,000 men.
Most people are diagnosed with the disease after the age of 40. The disease is most common in Scotland, Scandinavian countries, and northeast England.
Also, if you or someone in your family has another autoimmune disease, you are more likely to develop PBC . This suggests that there may be a genetic link.
What are the symptoms of (PBC) disease?
Many people with PBC do not have any symptoms in the early stages. It is only as the disease progresses that symptoms related to the bile ducts begin to appear. The first and most common symptoms seen in people with PBC are:
- Severe fatigue (about 65% of people)
- Itchy skin (about 55% of people)
These symptoms affect everyone differently. Some people may have them early in the disease, while others may have them later. They can also be more or less severe at any stage of the disease. There doesn't seem to be a big connection between how severe your symptoms are and how advanced the disease is. However, some research suggests that people who have severe symptoms early on may have a faster progression of the disease .
What are the complications of bile duct diseases?
In the early stages, you may not even notice that there is a problem with your bile ducts. But as the disease progresses, various complications begin to occur. Bile, which cannot flow properly, begins to leak into your bloodstream , which causes diseases. Also, when bile cannot reach the digestive system properly, digestion is also hampered. Because bile is essential for digesting food.
Later, the scarring of the liver can also affect the blood vessels that pass through the liver, leading to a condition called portal hypertension , which means high blood pressure in those veins.
1. Fat malabsorption
If your digestive system doesn't get enough bile, it can't properly break down and absorb the fats in your food. This reduced absorption of fats can cause the following:
- High blood cholesterol .
- Fat deposits under the skin .
- Fatty poop or diarrhea .
- Weight loss .
- Decreased levels of fat-soluble vitamins A, D, E, and K.
- Osteoporosis is a bone thinning disease caused by decreased absorption of fat-soluble vitamin D.
2. Portal hypertension
When the blood vessels that pass through the liver become blocked due to scarring, the aforementioned portal hypertension occurs, which means that the blood pressure in those veins and other veins connected to them increases. This portal hypertension can cause the following conditions:
- Enlarged liver .
- Enlarged spleen .
- Frequent colds and other infections (reduced immunity).
- Easy bruising and bleeding (low blood platelets - (thrombocytopenia) ).
- The appearance of red, spider-like blood vessels (spider angiomas) on the skin surface.
- Swollen or knotted veins in the oesophagus and abdomen (swollen veins - varices) .
- Gastrointestinal bleeding .
- Fluid accumulation in the abdomen (ascites) .
- Swelling (edema) of the lower body.
- Mental confusion sometimes occurs due to the accumulation of toxins in the blood.
What are the causes of (PBC)?
In PBC, your own immune system attacks the cells that line your liver's bile ducts . It's as if the immune system sees these cells as foreign invaders. This is what we call an autoimmune disease . For no apparent reason, the immune system destroys healthy cells, and there's no way to stop it. This ongoing inflammation causes scarring.
We don't really know exactly why these autoimmune diseases develop. However, there seems to be a genetic factor . Many people who develop autoimmune diseases have a family history of the disease, and they can develop more than one type of autoimmune disease.
There also seems to be an environmental factor . That is, perhaps something in the environment can trigger this disease. It could be a chemical that you are exposed to, or it could be an infection.
How is PBC diagnosed?
Your doctor will first ask you about your medical history and symptoms, perform a physical exam, and then take a blood sample to check for PBC.
These blood tests mainly look for specific antibodies associated with PBC, specifically an antibody called antimitochondrial antibody (AMA) . They also look for elevated liver enzymes , specifically alkaline phosphatase (ALP), which indicate liver damage.
If your test results are positive for PBC, your doctor will next want to look at images of your liver and biliary system . This can help rule out other causes of your symptoms and see how advanced the disease is. Usually, something as simple as an abdominal ultrasound is done first. But sometimes, a test like an MRI (magnetic resonance imaging) may be needed to get clearer images.
About 5% of people with PBC may have a negative AMA test, but they may have other symptoms. If this happens, your doctor may need to take a small piece of tissue from your liver (a liver biopsy) to confirm PBC. This is usually done at the bedside by inserting a needle into your liver. The small sample of tissue taken from the needle is sent to a lab to be examined under a microscope.
What are the treatments for Primary Biliary Cholangitis?
Doctors use the following to treat PBC:
Medication
PBC cannot be cured completely . However, medications can control the progression of the disease and improve your condition .
Ursodeoxycholic acid (UDCA) is a type of bile acid. It helps remove bile from the liver and reduce liver damage. This medicine works well for about half of people with PBC, especially in the early stages of the disease. For people who don't respond to UDCA, doctors sometimes recommend another type of bile acid called obeticholic acid .
Also, your individual symptoms can be treated with separate medications.
- For itchy skin: antihistamines (e.g., diphenhydramine - like Benadryl®), ultraviolet light therapy, or bile acid sequestrants such as cholestyramine may be given.
- Prevent side effects like vitamin deficiencies and osteoporosis: Vitamin supplements can help.
- Some people with fatigue may benefit from stimulants such as modafinil.
Surgery
If your condition does not improve with medication, and your liver function continues to decline, your doctor may put you on a waiting list for a liver transplant . People with PBC have very good results with liver transplant surgery.
Like other autoimmune diseases, PBC can recur after a liver transplant, but the rate of progression of the disease is much slower the second time around. Life expectancy after a liver transplant is normal.
What should you expect when living with this condition?
In most cases, Primary Biliary Cholangitis progresses slowly . Early detection and prompt treatment can prevent or at least delay the later stages of the disease and its complications.
Most people can control their symptoms well with medication. However, treating the fatigue that occurs with PBC can still be a challenge. Many people live for years without significant disruption to their daily lives due to PBC.
However, not everyone is diagnosed with PBC in its early stages. And some people develop a more aggressive form of the disease. Signs like severe fatigue and high bilirubin levels in the blood can indicate that the disease can quickly progress to liver failure. If you get to that stage, you will need a liver transplant to survive. But for those with PBC who have a successful liver transplant, the prognosis is very good.
How long can you live with (PBC)?
It usually takes between 15 and 20 years for the disease to reach the terminal stage.
The first stage, which means that even if you have been diagnosed with PBC, you may not have any symptoms for a long time. About half of people will start to develop symptoms within the next five to ten years. Once you do develop symptoms, the average life expectancy is about 10 years . 65% of people who have a successful liver transplant survive 10 years.
How do I take care of myself when I have (PBC)?
Maintaining a healthy diet and lifestyle will help keep your liver healthy for as long as possible.
For example:
- Stop smoking, drinking alcohol, and using drugs and medications improperly or for pleasure.
- Eat less processed and packaged foods and more whole foods.
- Reduce saturated fats and eat more healthy unsaturated fats.
- Take a brisk walk every day. Weight-bearing exercise can help maintain bone health.
Many people with Primary Biliary Cholangitis can live for years without any major problems. However, having a progressive, sometimes fatal disease can take a toll on you. Talk to your doctor about ways to reduce your physical, mental, and emotional stress . Also, ask about support networks that help people living with PBC. Remember, your overall health is much bigger than just the disease.
Take-Home Message
Primary Biliary Cholangitis (PBC) is a chronic disease that affects the bile ducts of the liver. It is thought to be caused by an autoimmune condition. Although symptoms may not appear at first, symptoms such as fatigue and skin itching may develop over time. As the disease progresses , it can lead to cirrhosis and liver failure.
However, with early detection and the use of medications such as (UDCA), the disease can be controlled . Some people may even need a liver transplant.
The most important thing is to seek medical advice and adopt a healthy lifestyle if you have these symptoms. You are not alone, don't be afraid to talk about this and get help.
` Primary Biliary Cholangitis, PBC, liver disease, bile ducts, cirrhosis, autoimmune, fatigue, skin rash, liver disease, bile ducts, cirrhosis, autoimmune
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