Primary sclerosing cholangitis (PSC) affects the liver and bile ducts. Symptoms and treatment vary from person to person.
PSC is a rare liver disease that affects the bile ducts. It’s progressive, which means it gets worse over time. Treatment includes taking certain medications, including antibiotics, undergoing procedures to address blockages, and — in cases of liver failure — receiving an organ transplant.
Here’s what you need to know about PSC, its symptoms, and the outlook with proper treatment.
The liver is an organ in your body that breaks down food into its essential nutrients (proteins, fats, etc.) and energy, vitamins, and minerals. It also works to remove toxins from the bloodstream and assists in the digestive process by making a fluid called bile.
More specifically, the bile ducts in the liver are responsible for transporting digestive juices (bile) from the liver to the small intestine and gallbladder. Bile helps break down fats and fatty vitamins in these organs and further aids digestion.
With PSC, inflammation causes damage in the form of scarring, also called fibrosis. The scarring narrows and eventually blocks the bile ducts. When the ducts are blocked, the bile cannot travel where it needs to go. This results in liver damage.
There are four stages of PSC:
- Cholangitis or portal hepatitis: With cholangitis, scarring is limited to the portal areas of the liver.
- Periportal fibrosis or periportal hepatitis: Areas of scarring extend beyond the portal areas but do not yet connect.
- Septal fibrosis, bridging necrosis (or both): Areas of scarring begin to connect to one another.
- Biliary cirrhosis: Scarring forms “honeycomb” shapes and becomes more widespread.
You may not have any symptoms or only mild signs in the beginning stages, so any symptoms you’re experiencing do not necessarily relate to the stage of PSC.
That said, symptoms tend to get more severe with each stage and continued damage to the liver.
Doctors don’t know exactly what causes PSC. And what causes it may be different for different people.
What they do know is that:
- Some 70% of people who are affected are men, primarily young and middle-aged men.
- Around 80% of people with PSC also have inflammatory bowel disease.
- Several factors may increase your risk, like having certain viral or bacterial infections or a family history of the condition.
You may not have symptoms in the early stages of PSC. Some people may be asymptomatic or only have mild symptoms for the first few years. If you do have symptoms, they may come and go and get worse as the condition progresses.
Possible symptoms include:
If you’re experiencing any concerning symptoms, make an appointment with a primary care doctor. They may refer you to a liver specialist called a hepatologist for more detailed testing and treatment.
Signs of PSC may show up as abnormal results on liver blood tests. A more concrete diagnosis can be made after an liver MRI or a test called a cholangiography, where contrast dye is injected into the bile ducts and then viewed on an X-ray.
To treat PSC, doctors will first personalize treatment by focusing on the symptoms a person is experiencing. The main goal of treatment is not to cure the condition but to slow its progression.
Treatment options include:
- Medications: Your doctor may prescribe antibiotics to prevent infection or manage inflammation. Other medications include cholestyramine to help manage skin itching and bisphosphonates to protect bone mass and treat osteoporosis.
- Surgery: You may have endoscopic surgery to remove blockages in the bile ducts or widen narrowed ducts.
- Lifestyle changes: You may need to reduce or avoid your alcohol intake and quit smoking to further protect your liver. Eating a balanced, nutritious diet and taking supplements may also help address nutritional deficiencies.
- Vaccination: Your doctor may recommend getting vaccinated against both hepatitis A and B to provide extra protection for your liver.
A liver transplant may be necessary in the later stages of PSC. This is usually around 10 years after diagnosis.
Examples of later-stage complications might include:
- itching that is difficult to manage
- recurrent bacterial cholangitis
- end stage liver disease
- liver failure
Liver transplants are generally effective in treating PSC, and many people go on to have a full, healthy life. However, it’s also possible for PSC to come back, even with a transplant.
The National Health Service (NHS) in the United Kingdom provides universal healthcare. The NHS generally pays for both inpatient and outpatient hospital care, which may include liver procedures and transplants.
Still, there is no legislation or “absolute right” to specific care of any kind. It’s important to check with your hospital for details on the coverage provided for you and your procedure.
The average time a person waits for a liver transplant in the United Kingdom is between 5 and 7 months. This time may be shorter or longer depending on organ availability and your health status.
In the U.S., many insurance plans cover the basic treatments and prescription medications that might come with PSC. Of course, insurance plans vary, so it’s important to know what your plan formulary allows. Most insurance companies also cover liver transplants, though it’s important to check with your carrier to verify that coverage and your out-of-pocket costs.
Without treatment, people with PSC may experience:
- liver failure
- frequent infections
- certain cancers
Both bile duct cancer (cholangiocarcinoma) and gallbladder cancer are two types of cancer that are more likely to occur in people with PSC.
Again, this condition is progressive, so it tends to worsen over time. Treatment may slow the progression and treat symptoms, but a liver transplant is often necessary.
The outlook for people who have undergone a liver transplant for PSC is favorable. Around 80% survive beyond 2 years after surgery with a good quality of life.
PSC is a rare, progressive liver disease that affects the bile ducts. Symptoms can vary in intensity and may include fatigue, itching, and yellowing skin and eyes.
If you’re experiencing any symptoms that concern you, make an appointment with a primary care doctor. They may refer you to a hepatologist for tests and treatments.
There are various treatments for PSC, depending on the stage and symptoms you’re experiencing. Some people with PSC may need a liver transplant around 10 years after their diagnosis. After a transplant, many people go on to have a good quality of life.