Primary sclerosing cholangitis (PSC) is a rare liver disease that affects the bile ducts.
PSC is 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 and its symptoms, as well as 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 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 is what helps break down fats and fatty vitamins in these organs and further aids with 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.
- Cholangitis or portal hepatitis: 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 have no symptoms or only mild symptoms in the beginning stages, so any symptoms you’re experiencing do not necessarily relate to what stage you are in.
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:
- 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.
- A number of 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:
No two people with PSC receive the same treatment. Instead, doctors personalize treatment and focus 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 control inflammation. Other medications include cholestyramine to help control 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 ducts that have been narrowed.
- 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 further protect your liver.
A liver transplant may be necessary later stages of PSC. Examples of later-stage complications might include:
Experts say liver transplants are effective, but it’s possible for PSC to come back even with a transplant.
The National Health Service (NHS) provides universal healthcare in the United Kingdom. The NHS generally pays for both inpatient and outpatient hospital care, which may include liver procedures and transplants.
Still, there’s no legislation or “absolute right” to any specific care of any kind. It’s important to check with your hospital for details on coverage provided for you and your procedure.
The average time a person waits for a liver transplant in the United Kingdom is between 3 and 4 months. This time may be shorter or longer depending on organ availability and your health status.
As to the United States, 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
Again, this condition is progressive, so it tends to get worse over time. Treatment may slow the progression and treat symptoms, but a liver transplant is often needed.
The outlook for people who have undergone a liver transplant for PSC is good. Around 80% survive beyond 2 years after surgery.
If you’re experiencing any symptoms that concern you, make an appointment with a primary care doctor. They may refer you to a liver specialist called a hematologist for more detailed testing and treatment.
Many people with PSC may need a liver transplant around 10 years after their diagnosis. The good news is that after a transplant, you may go on to live a healthy, full life.