Primary sclerosing cholangitis (PSC) is a rare liver disease that affects the bile ducts.
PSC is progressive and gets worse over time. Treatment includes taking certain medications, undergoing procedures to address obstructions, and receiving an organ transplant.
Here is what you need to know about PSC and its symptoms.
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.
The bile ducts in the liver are the main route for bile to travel from the liver to the small and gallbladder. ble helps break down fat and vitamins in the body.
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:
- Men are the majority of people who are affected.
- PSC has a high incidence of inflammatory bowel disease.
- A family history of the condition, as well as having certain viral orbacterial infections, may increase your risk.
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:
Two people with PSC are treated the same. Doctors focus on the symptoms a person is experiencing instead of the treatment. The main goal of treatment is to slow the progression of the condition.
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:
- difficult to manage itching
- There are recurrent cholangitis.
- End-stage disease.
- The bile duct is failing
Experts say liver transplants are effective, but it’s possible for PSC to come back even with a transplant.
Universal healthcare is provided by the National Health Service in the United Kingdom. The National Health Service pays for both in and out of hospital care, which may include 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.
The basic treatments and prescription medications that might come with PSC are covered by many insurance plans in the United States. It is important to know what your plan allows. It is important to check with your insurance company to make sure that you are covered for the transplant and your out-of-pocket costs.
People with PSC may not experience treatment.
- The bile duct is failing
- There are frequent infections.
- Certain types of 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.