Primary Sclerosing Cholangitis, or PSC for short, is a rare disease that affects the bile ducts in the liver. These ducts are tiny tubes that carry bile, a liquid made by your liver to help digest food. In people with PSC, these bile ducts get inflamed (swollen) and scarred, making it hard for bile to flow normally. Over time, this can damage the liver and cause it to stop working properly.
PSC is a serious, long-lasting disease that can get worse over time. While some people may not feel sick for many years, others may develop problems more quickly. Even though doctors are still trying to understand exactly what causes PSC, they’re working hard to find ways to treat it.
What Does the Liver Do?
To understand how PSC affects the body, it’s helpful to know what the liver and bile ducts do. Your liver is a large organ that has many important jobs, such as:
- Removing harmful chemicals (toxins) from your blood.
- Producing bile, which helps break down fats in your food.
- Storing vitamins and energy.
Bile is made in the liver and flows through the bile ducts into your small intestine, where it helps digest food. But when the bile ducts become blocked or inflamed, like in PSC, bile can get stuck in the liver. This can cause serious damage to the liver over time.
What Causes PSC?
Even though scientists aren’t completely sure what causes PSC, they do know that it’s likely connected to a problem with the immune system. In people with PSC, the immune system, which normally protects the body from harmful invaders like bacteria or viruses, starts attacking the bile ducts instead. This is called an autoimmune disease.
Most people who have PSC also have another autoimmune disease, usually something called inflammatory bowel disease (IBD), which includes conditions like ulcerative colitis or Crohn’s disease. This suggests that there’s a link between problems in the digestive system and PSC.
Symptoms of PSC
One of the tricky things about PSC is that it often doesn’t cause symptoms right away. Some people might not know they have it until they go to the doctor for something else. However, as the disease gets worse, symptoms can appear. Here are some common signs of PSC:
- Fatigue (Tiredness): Many people with PSC feel very tired all the time, even if they’ve had plenty of rest.
- Itching: Some people develop intense itching, especially on their hands and feet, which is caused by a buildup of bile in the body.
- Jaundice: This happens when the skin and the whites of the eyes turn yellow. It happens because the liver can’t process a substance called bilirubin.
- Pain: Some people with PSC feel pain in the upper right part of their abdomen, which is where the liver is located.
How Do Doctors Diagnose PSC?
Since PSC is a rare disease and its symptoms can be similar to other liver problems, it can be difficult to diagnose. Doctors usually use a combination of tests to figure out if someone has PSC.
- Blood Tests: Doctors will check for signs of liver damage by measuring certain chemicals in the blood, like alkaline phosphatase. If levels are too high, it could mean there’s a problem with the bile ducts.
- Imaging Tests: To get a better look at the bile ducts, doctors might use special scans like MRI or CT scans. These pictures show whether the bile ducts are narrowed or blocked.
- Liver Biopsy: In some cases, doctors will take a small piece of the liver to look at under a microscope. This can help them see how much damage has been done.
How Is PSC Treated?
Unfortunately, there is no cure for PSC at the moment. However, doctors can help manage the symptoms and slow down the damage to the liver. Here are some common ways to treat PSC:
- Medication: Medicines can help reduce itching, treat infections, and control problems with digestion. For example, a drug called cholestyramine can help relieve itching by reducing bile buildup.
- ERCP: Sometimes, doctors will use a procedure called ERCP to open up blocked bile ducts. This can help bile flow more freely, which might relieve some symptoms.
- Liver Transplant: If PSC becomes very advanced and the liver is badly damaged, a liver transplant may be needed. In this surgery, the damaged liver is replaced with a healthy one from a donor. Liver transplants have been very successful in helping people with PSC live longer, healthier lives.
Living with PSC
Living with PSC can be challenging, but many people with the disease can still live full lives for many years. Patients with PSC often need to visit the doctor regularly to check on their liver health and manage their symptoms. Because PSC progresses differently in each person, doctors will create a specific treatment plan based on how the disease is affecting the individual.
Some people find it helpful to join support groups to connect with others who understand what they’re going through. Support groups provide a safe space to share experiences, advice, and emotional support.
What Research Is Being Done?
Scientists are working hard to learn more about PSC and to find new treatments. Research is focused on understanding what causes the immune system to attack the bile ducts and figuring out how to stop or slow down the inflammation. There are clinical trials testing new medications that could reduce scarring in the bile ducts or prevent the liver from getting worse.
Although PSC can’t be cured yet, there is hope that better treatments will become available in the future. Ongoing research may eventually lead to a cure, giving patients with PSC a brighter outlook.
In Summary: Primary Sclerosing Cholangitis is a rare liver disease that affects the bile ducts, causing scarring and liver damage over time. While doctors are still trying to understand what causes PSC, they believe it’s linked to autoimmune problems. Symptoms of PSC can include tiredness, itching, yellowing of the skin, and abdominal pain. Though there’s no cure yet, treatments can help manage symptoms and slow the disease’s progression. In some cases, a liver transplant may be necessary. Scientists are continuing to research PSC to find better treatments and, hopefully, a cure in the future.