Primary Sclerosing Cholangitis (PSC) is a chronic or long-term disease that slowly damages the bile ducts inside and outside of the liver. Bile is a digestive liquid that is made in the liver that travels through the bile ducts to the gall bladder and the small intestine where it help digest fats and fatty vitamins.
PSC inflammation of the bile ducts leads to scarring and narrowing of the ducts over time. As the scarring increases, the ducts become blocked. Bile then builds up in the liver and damages the liver cells. The scarring can spread in the liver causing cirrhosis and liver failure.
What causes PSC is not known but a person’s genes, immune system problems, bacteria, and viruses may play a role in the development of the disease. PSC is linked to inflammatory bowel disease (IBD). About three out of four people with PSC have ulcerative colitis. Most people who have PSC are adults but PSC can also be found in children. PSC is more common in men than women, and having a family member may increase a person’s risk for developing PSC.
The main symptoms of PSC are:
- Getting very tired (fatigued)
- Yellowing of the skin or whites of the eyes.
- Chills and fever
- Abdominal pain
- Weight loss
The first step is to do a blood test to check the liver enzymes.
The doctor can confirm the diagnosis using several tests:
- Endoscopic retrograde cholangiopancreatography (ERCP) – uses an endoscope that goes down the mouth and into the duodenum to reach an area in the digestive tract where dye can be put into the bile ducts. X-rays are then taken. ERCP can also take a tissue sample or treat blocked ducts.
- Percutaneous transhepatic cholangiography – a needle is put into the skin and a thin tube goes into a duct in the liver. A dye is placed through this tube so X-rays can be taken.
- Magnetic resonance cholangiopancreatography (MRCP) – uses MRI radio waves as magnets to look at organs and tissues inside the body. This is safe and painless.
- Other testing may include ultrasound exams and a liver biopsy.
The main treatment of PSC is to relieve the symptoms and try and keep it from getting any worse. Medical treatment may include:
- Medicines to relieve itching
- Antibiotics to treat infections
- Vitamin supplements
- Opening the bile duct that is blocked using a ballon dilation and stent.
- Bile duct surgery
- Liver transplant
Steps to care for your liver if you have PSC:
- Get vaccinated against hepatitis A and B.
- Use care with using chemicals and home or work.
- Choose a healthy diet of fruits, vegetables and whole grains.
- Maintain a healthy weight
- Follow directions when taking over-the-counter medicines.
- Don’t drink alcohol or smoke.