Sometimes we feel tired for no reason, we get a slight ache in the upper right side of our stomach, or maybe we just feel like we're scratching ourselves. Although we don't pay much attention to these things in our daily lives, sometimes these can be signs of a more serious condition inside our body. Today we are going to talk about a condition that is a bit complicated, but it is very important for everyone to be aware of. That is Primary Sclerosing Cholangitis , or PSC for short.
What is primary sclerosing cholangitis (PSC)?
Simply put, PSC is a disease that occurs in the bile ducts in our body. As you may remember, bile is produced in the liver. This bile helps in digestion. So, there is a system of small tubes that carry this bile from the liver to the intestines. We call those tubes the bile ducts.
In PSC, there is chronic inflammation , or swelling, in these bile ducts. Doctors also call this cholangitis . When this inflammation continues for a long time, scarring begins to form in the bile ducts. This scarring causes the bile ducts to become hard and narrow. This is called sclerosis .
Imagine a water pipe that is clogged with dirt and narrows, making it difficult for water to flow through, right? Similarly, when the bile ducts become narrow, the flow of bile is restricted. This is called a biliary stricture . So, when bile cannot flow properly, it starts to back up into the liver. This accumulation of bile in the liver (cholestasis) gradually damages the liver.
Is this PSC situation serious?
Yes, PSC is actually a fairly serious disease . You may not have any symptoms in the early stages. But it is a progressive disease. When bile is blocked in the bile ducts (cholestasis), some of the toxins in the bile can build up in the blood. Then you start to feel uncomfortable. Also, this causes ongoing damage to the liver.
Typically, this damage can lead to complete liver failure after about 10 to 15 years. As you know, we cannot live without a liver. Although current treatments can control the symptoms, there is still no cure for PSC. The only solution is a liver transplant .
What is the difference between primary and secondary sclerosing cholangitis?
The word "primary" here means that the disease is the primary cause of the inflammation and scarring of the bile ducts. That is, the condition is caused by this disease alone, without any other cause.
However, in a condition called secondary sclerosing cholangitis , the inflammation (cholangitis) and scarring (sclerosis) of the bile ducts occur as a result of another cause. That is, the condition is caused by another disease or event. Here are a few examples:
- Injury to the bile ducts (for example, during surgery).
- Infections in the bile ducts.
- Anti-cancer treatment (Chemotherapy).
- Gallstones blocking the bile ducts.
- Recurring pancreatitis.
What is the difference between primary biliary cirrhosis (PBC) and PSC?
The disease, formerly known as Primary Biliary Cirrhosis, is now called Primary Biliary Cholangitis (PBC) .
Both PBC and PSC are progressive diseases that affect the liver, affecting the bile ducts. The symptoms and effects of both are very similar. Both diseases cause the bile ducts to gradually weaken. When the bile ducts become damaged, scarred, and narrowed, the flow of bile becomes blocked. The bile then backs up into the liver, further damaging the liver. This can lead to scarring of the liver and eventually cirrhosis of the liver .
But there are several key differences between these two diseases:
- PBC only affects the bile ducts inside the liver (intrahepatic). PSC can affect both the bile ducts inside the liver and the bile ducts outside the liver (extrahepatic).
- PBC is more common in women (10:1 ratio), while PSC is more common in men (2:1 ratio).
- About 80% of PSC patients have a pre-existing inflammatory bowel disease (IBD) , especially ulcerative colitis . However, this is not a significant association with PBC.
- There is an increased risk of bile duct cancer with PSC. There is no such high risk with PBC.
- Ursodeoxycholic acid (UDCA) is used to treat PBC, but there is currently no specific treatment for PSC.
How common is this condition called PSC? Who usually gets it?
PSC is a very rare disease . Worldwide, it is estimated that only one in ten thousand people have this disease.
This disease is twice as common in men as in women. The disease is usually diagnosed around the age of 40. About 80% of people with PSC also have IBD (Inflammatory Bowel Disease) , especially Ulcerative Colitis . If someone in the family has this disease, others are at risk of developing it too.
What causes PSC?
The exact cause of PSC is not yet known, but it is thought to be caused by a combination of factors. These include:
- Genetics
- Environmental factors
- Immune cells
Doctors think PSC may be a type of autoimmune disease . This means that our body’s own defense system, the soldiers that are supposed to protect us from disease, mistakenly starts attacking our own healthy cells. Inflammation is one of the weapons of our immune system. It’s usually temporary, acting against an invader. But if inflammation becomes chronic, it’s usually a sign of disease.
Doctors have also observed that people with PSC are more likely to develop other autoimmune diseases. For example:
- Inflammatory bowel diseases
- Celiac disease
- Thyroid disease
- Type 1 diabetes
- Autoimmune hepatitis
- Autoimmune pancreatitis
It is thought that this type of autoimmune response may occur when people with certain genetic traits are exposed to certain environmental factors (e.g., exposure to toxic chemicals).
What are the symptoms of PSC?
As PSC progresses, symptoms change. About 50% of people may have no symptoms at all when the disease is diagnosed. PSC is often discovered incidentally, during tests for another condition. Early symptoms are usually not very obvious. They include:
- Fatigue
- Upper right quadrant abdominal pain
- Itchy skin (pruritus)
Symptoms that may occur as the disease progresses include:
- Swollen abdomen
- Enlarged liver
- Enlarged spleen
- Jaundice - This means yellowing of the whites of the eyes and skin.
- Fever
- Unintended weight loss
What are the complications of severe PSC?
Primary sclerosing cholangitis is a slowly progressive disease, but as your bile ducts and liver become scarred and their function deteriorates, various complications can occur.
Metabolic diseases
If your bile ducts are significantly blocked, they can't properly pump bile into your small intestine. Bile is essential for digesting food, especially fat, and for absorbing fat-soluble vitamins (A, D, E, and K). Without bile, your digestive system can't properly process fat, and it can't absorb these essential vitamins from food. This can lead to additional complications:
- Fatty stools and diarrhea
- Malabsorption and malnutrition
- Easy bruising and bleeding (due to vitamin K deficiency)
- Bone weakness - Osteomalacia and osteoporosis (due to vitamin D deficiency)
- Poor night vision (due to vitamin A deficiency)
Portal hypertension
As cirrhosis progresses, blood flow through the liver decreases. This increases pressure in the portal vein, which runs through your digestive system. This pressure can cause other veins in your esophagus and stomach to enlarge and burst. This can lead to internal bleeding .
Infections
Blocked bile ducts can often become infected, causing conditions such as fever, stomach pain, and blood poisoning (sepsis).
Cancer
As PSC becomes more severe, the risk of developing several types of cancer increases:
- Bile duct cancer (cholangiocarcinoma) - Risk between 5% and 20%.
- Gallbladder cancer
- Liver cancer (hepatoma)
- Colorectal cancer - if you also have IBD.
How is PSC diagnosed?
Most often, the disease is discovered by chance, when testing for something else. About 50% of people may not have any symptoms when the disease is diagnosed. Early signs of the disease can be seen with a blood test or an imaging test. Scans of the bile ducts with PSC show special features. If a blood test shows high levels of an enzyme called alkaline phosphatase , or if there are certain antibodies that indicate an immune response to the bile ducts, it can be a sign of PSC. An increase in white blood cells is usually a sign of a liver infection.
To confirm the diagnosis, your doctor may suggest several more specific tests:
- Liver function test (LFT) : These blood tests look for elevated levels of specific enzymes in the liver. Elevated levels of alkaline phosphatase may indicate PSC.
- Magnetic resonance cholangiopancreatography (MRCP) : This is an MRI (Magnetic Resonance Imaging) scan. It can take detailed pictures of your biliary system (liver, gallbladder, and bile ducts). This is the first scan to diagnose PSC. This is because it is non-invasive and does not involve radiation exposure. However, sometimes, if the disease is early or not very severe, it may not be able to detect it accurately. Then another scan may be needed.
How is PSC treated?
Currently, there is no treatment that can stop or reverse the progression of primary sclerosing cholangitis. However, symptoms and complications can be treated directly. For example, your doctor may prescribe things like:
- Medications to treat itchy skin (pruritus)
- Supplements to treat vitamin deficiencies
- Antibiotics to treat infections
Your doctor will keep a close eye on your liver and bile ducts. As the disease progresses, they may occasionally open up a blocked bile duct. This can be done through a test called ERCP (endoscopic retrograde cholangiopancreatography) . This is a procedure that allows you to see into your bile ducts without surgery.
ERCP is a type of endoscopy . It involves examining your organs using an endoscope, a long, flexible tube with a small camera and light on it. After you are anesthetized, your doctor will insert this tube down your throat and into your abdomen. Small instruments can be passed through this tube to treat your bile ducts. The technician may use a balloon to widen your bile duct, or insert a stent to keep it open. If ERCP cannot reach the blockage, a procedure called `percutaneous trans-hepatic cholangiography (PTHC)' is sometimes used.
But this is only a temporary solution. Over a period of 10 to 20 years, primary sclerosing cholangitis gradually worsens, the liver disease becomes worse, and eventually liver failure occurs. Your doctor will monitor the damage to your liver and decide when to consider a liver transplant . You also need to meet certain criteria to be on the waiting list for a liver transplant.
How long can you live with PSC?
Once diagnosed, the average life expectancy varies between 10 and 20 years. A liver transplant can give a new lease of life. However, in 15% to 20% of cases, PSC can come back even after a liver transplant. If that happens, the new liver can also fail. In such cases, the average life expectancy is about nine months.
Another factor that can affect life expectancy is cancer. If you develop cancer as a complication of PSC, you may not be a good candidate for a liver transplant. In carefully selected cases, doctors may first treat the cancer with radiation or chemotherapy, and then try a liver transplant.
How should I take care of myself if I have this disease?
Making lifestyle changes can help manage the fatigue that is common with PSC and prevent further liver damage. For example:
- Avoid alcohol completely.
- Eat less processed foods and more whole foods.
- Control stress.
- Sleep well.
- Do some exercise every day.
Primary sclerosing cholangitis is a rare, unpredictable, and preventable disease. It progresses slowly, and often has no symptoms in the early stages. But if you see your doctor regularly and get routine screenings, the disease can be detected before it starts to affect your life. That's when you can make lifestyle changes that can help keep your liver healthy for longer. You'll also be referred for long-term medical care, which means regular checkups and interventions if needed.
Finally, things to remember
Okay, so I hope what we've talked about about PSC has helped you remember some of the most important things. It's a bit of a complicated situation, but it's very important to be aware of it.
- PSC is a disease that affects the bile ducts and gets worse over time. It can damage the liver.
- There may be no symptoms in the early stages, so it is important to have regular medical checkups.
- There is currently no cure for this disease, but there are treatments to control symptoms and improve quality of life.
- Liver transplantation is the main solution in severe cases.
- Following a healthy lifestyle helps protect the liver.
If you have any further questions about PSC, or if you think you have any of these symptoms, be sure to see a doctor for advice . Don't worry, there are things you can do to manage it if it's caught early.
` Primary Sclerosing Cholangitis, PSC, Bile Duct Disease, Liver Disease, Jaundice, Stomach Pain, Primary Sclerosing Cholangitis
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