Primary sclerosing cholangitis (PSC) is a relatively rare chronic disease that causes inflammation of your bile ducts and liver, which can lead to liver damage or failure. The only complete cure for PSC is a liver transplant. The objective of PSC treatment is to slow the progression of damage to the liver and prevent liver failure.
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What Is Primary Sclerosing Cholangitis?
Primary sclerosing cholangitis (PSC) is a chronic disease in which your bile ducts and liver get inflamed and swollen.
Your bile ducts are tubes that carry bile, a fluid produced by the liver, from your liver to your small intestine and gallbladder. PSC causes the bile ducts to harden and become blocked over time, resulting in the accumulation of bile in the liver. This buildup of bile causes liver damage and can even lead to liver failure.
Damage can occur quickly or over many years, and doctors can't reverse it. Treatment focuses on slowing down liver damage for as long as possible.
How common is primary sclerosing cholangitis?
PSC is a relatively rare disease. About 1 in 10,000 people have PSC. Doctors diagnose this disease in about 1 in 100,000 people per year worldwide.
What causes primary sclerosing cholangitis?
Doctors don't know exactly what causes PSC. The disease likely results from a combination of genetic and environmental factors.
Primary sclerosing cholangitis risk factors
The following factors may increase your risk of developing PSC:
- Age — Most people are diagnosed with PSC between the ages of 30 and 50.
- Autoimmune disorders — Especially type 1 diabetes.
- Gender — Men are twice as likely as women to develop PSC.
- Genetics.
- Inflammatory bowel disease (IBD) — About 60% to 80% of people with PSC also have IBD, especially ulcerative colitis, a severe inflammation of the intestines that causes ulcers and stomach pain, or Crohn’s disease, which can cause inflammation in any part of the gastrointestinal tract.
Primary sclerosing cholangitis complications
Left untreated, PSC can lead to liver failure, which would necessitate a liver transplant. Liver failure that cannot be helped through transplant can lead to death. Without a liver transplant, the average rate of survival upon diagnosis is about 10 years.
Without adequate bile, the body can’t digest fat-soluble vitamin D, which helps to protect bones. This can lead to osteoporosis, a condition characterized by the thinning of bones, which leaves them prone to fractures.
PSC can also lead to cholangitis, or inflammation of the bile ducts, gallstones, and narrowing or obstruction of the bile duct. About 10% to 20% of people with PSC will also develop cholangiocarcinoma, or bile duct cancer, as a result. Gallbladder cancer may also occur.
How can I prevent primary sclerosing cholangitis?
There is currently no way to prevent someone from getting PSC. This might change in the future if researchers can decode the genetics of this disease.
What Are the Signs and Symptoms of Primary Sclerosing Cholangitis?
Even though PSC is a serious disease, about 50% of people who have it don't have symptoms right away. As the disease worsens, symptoms can come and go.
Symptoms of PSC include:
- Chills.
- Fatigue.
- Night sweats.
- Pain in the upper right part of the belly.
- Pruritus — Extreme itching.
- Unexplained weight loss.
- Yellowed skin or eyes, known as jaundice.
When should I see a doctor about primary sclerosing cholangitis?
You should see your doctor if you have any symptoms of PCS. These symptoms could have many causes, however, so expect your doctor to run a range of tests to confirm a diagnosis.
How Do You Diagnose Primary Sclerosing Cholangitis?
Your doctor will order tests to help diagnose PSC, including:
- Blood tests.
- Endoscopic ultrasound and endoscopic retrograde cholangiopancreatography (ERCP). This test combines endoscopy with dye and X-ray technology to view the bile ducts.
- Endoscopy — Uses a scope to see inside a person's digestive system.
- FibroScan® — A noninvasive liver ultrasound to determine how much fibrosis (excessive connective tissue) your liver has.
- Liver biopsy — Surgery to remove a small piece of liver to look at it under a microscope.
- MRI scans.
Doctors may suspect PSC when routine blood tests or liver function tests have abnormal results, even if you don't have symptoms. It is important to diagnose PSC as early as possible to prevent liver failure.
How Do You Treat Primary Sclerosing Cholangitis?
If you have PSC, your doctor will track your liver health closely to find and treat any new problems right away. They will also treat any infections with antibiotics and manage symptoms like pain or jaundice. Your doctor may recommend that you get a colonoscopy.
There is currently no established treatment plan for PSC, but studies are underway to determine if ursodeoxycholic acid, a medication used to treat liver conditions, can help improve symptoms. Cholestyramine, a bile acid sequestrant, has also been shown to be effective in reducing PSC symptoms.
Lifestyle changes
Your doctor may also recommend certain lifestyle changes to help manage PSC, including:
- Avoiding alcohol.
- Eating a variety of fruits, veggies, and whole grains.
- Getting vaccines for hepatitis A and hepatitis B.
- Maintaining a healthy weight.
- Quitting smoking.
- Regular exercise and movement.
If your bile ducts become blocked, your doctor may need to do minor surgery.
Surgery
Types of surgery to open the ducts so that bile will no longer build up in your liver include:
- Endoscopy — Uses a scope to open the bile ducts.
- Esophageal variceal banding — Wraps a small band around blood vessels in the liver to prevent internal bleeding.
- Paracentesis — Uses a hollow needle to withdraw extra fluid through the abdominal wall.
- Thoracentesis— Uses a hollow needle to withdraw fluid from the chest or lungs.
- Transjugular intrahepatic portosystemic shunt (TIPS) — Uses a small coil or stent inserted into a vein in the liver to reduce liver hypertension or bleeding risk.
In cases where PSC causes severe cirrhosis (liver failure), you may need a liver transplant. Transplant provides the only known cure for PSC.
How long does it take to recover after primary sclerosing cholangitis treatment?
PSC is a serious chronic condition for which there is no cure, other than liver transplantation. That means you will be dealing with the effects of this disease for the rest of your life.
UPMC liver disease experts can help you slow the progression of your disease. If you have a liver transplant, you and your health team will need to manage a suppressed immune system and related complications for the rest of your life.
