Primary Sclerosing Cholangitis (PSC)

What is primary sclerosing cholangitis?

Primary sclerosing cholangitis, or PSC, is a rare, long-term liver disease that damages your larger bile ducts located inside and outside your liver. The bile ducts are part of the biliary tree. They carry bile from your liver to your small intestine, so the bile can help you digest fats and absorb vitamins. 

PSC causes inflammation of the bile ducts. The ducts become narrow and bile can’t flow normally. When bile backs up, it harms your liver cells. Over time, it can cause cirrhosis and liver failure. PSC is a chronic condition, so you need regular medical care and monitoring.

Causes and risk factors

Experts don’t know exactly what causes PSC but it involves an immune system response.

These issues may play a role:

• Immune system dysfunction
• Genetic factors
• Environmental or gut-related triggers

Risk factors may include:

• Having inflammatory bowel disease, especially ulcerative colitis
• Having a family history of liver or autoimmune disease, including autoimmune hepatitis
• Being male, since PSC is more common in men 
• Being between age 30 and 60, when most cases are diagnosed

Not everyone with these risk factors develops PSC.

Signs and symptoms of PSC

At first, you may have mild symptoms or no symptoms at all. Many people find out they have PSC when routine blood tests show abnormal liver results.

As the condition gets worse, you may notice more symptoms of primary sclerosing cholangitis.

Common early symptoms include:

• Fatigue
• Itchy skin
• Abdominal pain or discomfort on the right side
• Fever or chills if bile ducts get infected

As PSC gets worse, you may notice:

• Yellowing of the skin or eyes (jaundice)
• Dark urine
• Pale or clay-colored stools
• Unexplained weight loss
• Swelling in the belly or legs from fluid buildup

Because bile helps your body absorb fat and fat-soluble vitamins, you may have diarrhea, greasy or fatty stools and signs of low levels of vitamins A, D, E or K.

How PSC is diagnosed

To diagnose PSC, your provider will ask about your medical history and any symptoms. They will perform a physical exam to see if your liver or spleen is enlarged. 

Tests that can help diagnose primary sclerosing cholangitis and rule out other causes of bile duct problems include:

• Blood tests that show a cholestatic pattern
• Imaging with magnetic resonance cholangiopancreatography (MRCP) a specialized type of magnetic resonance imaging (MRI)
• Endoscopic retrograde cholangiopancreatography (ERCP) in selected cases, usually to treat a narrowed area (stricture) or collect samples
• Liver biopsy when the diagnosis is unclear or to see how much liver damage there is

Your care team may also:

• Order a colonoscopy to look for inflammatory bowel disease
• Test for specific antibodies
• Check vitamin levels and bone health

Treatment and care

There is no medicine that can stop or reverse PSC, so treatment focuses on managing symptoms, preventing and treating complications and monitoring the disease. 

Your care plan may include:

• Medication to relieve itching and treat infections
• Procedures that open narrow bile ducts so bile can flow better
• Regular blood tests and imaging to check liver function and bile duct changes
• Vitamin supplements 
• Regular bone density testing, since osteoporosis is common in long-term PSC
• More frequent colonoscopies, especially if you have IBD
• Regular ultrasounds to check for gallbladder polyps and periodic MRI/MRCP to evaluate for bile duct cancers
• Lifestyle steps to protect your liver, such as avoiding alcohol

For advanced PSC or liver failure, liver transplant is the only effective treatment. 

Complications and advanced disease

PSC can lead to serious complications over time, including:

• Bile duct infections
• Liver fibrosis or scarring and cirrhosis 
• Liver failure
• High blood pressure in the veins that connect to the liver, called portal hypertension
• Internal bleeding
• Fluid buildup in the abdomen
• Osteoporosis
• Problems from not absorbing fat soluble vitamins well
• Higher risk of cancers of the bile ducts, gallbladder and, if cirrhosis is present, cancer of the liver 

When to see a specialist

You should see a gastroenterologist (digestive system specialist) or hepatologist (liver specialist) if:

• Your provider thinks you may have PSC or has told you that you have PSC
• Your liver blood tests stay abnormal over time
• You have inflammatory bowel disease and new signs of liver disease
• Your symptoms are getting worse or you have complications

PSC is a complex disease and long-term specialty care is important.

Find care at Banner Health

You can turn to Banner Health for expert care for primary sclerosing cholangitis and other complex bile duct and liver conditions. Our specialists work together to:

• Evaluate and diagnose PSC and other liver diseases
• Treat bile duct problems with endoscopic and interventional procedures
• Provide supportive care for nutrition, bone health and quality of life

Connect with a Banner Health liver specialist who understands PSC and can guide your care.

Learn about primary biliary cholangitis (PBC).