Primary Sclerosing Cholangitis

Diagnosis

To diagnose primary sclerosing cholangitis (PSC), your healthcare professional may use several tests and procedures:

• Liver function blood tests. These check protein and enzyme levels, such as alkaline phosphatase, to assess liver function.

• MRI of the bile ducts. Magnetic resonance cholangiopancreatography (MRCP) produces detailed images of the liver, bile ducts, gallbladder, and pancreas.

• X-rays of the bile ducts. A cholangiogram uses dye to highlight the bile ducts on X-ray images. The dye may be delivered through the digestive tract using endoscopic retrograde cholangiopancreatography (ERCP) or injected directly into the liver.

• Liver biopsy. A small sample of liver tissue is removed using a needle and examined for inflammation, scarring, and bile duct changes. Biopsy is useful when imaging is inconclusive or if there is concern about overlap with autoimmune hepatitis.

• Colonoscopy. A flexible tube with a camera allows your healthcare professional to examine the colon, perform biopsies, and screen for inflammatory bowel disease, cancer, or other irregularities. Colonoscopy is also part of regular monitoring for PSC patients.

Treatment

There are no medical therapies that change the course of PSC. Treatment focuses on symptom management, preventing complications, and monitoring liver damage.

Ursodiol
Low doses of ursodiol (ursodeoxycholic acid) may improve or stabilize liver function temporarily. It does not prevent disease progression but may reduce itching.

Treatment for itching

• Antihistamines may relieve mild itching and improve sleep.

• Bile acid sequestrants, such as cholestyramine and colestipol, bind bile acids to reduce moderate to severe itching. They may cause constipation and vitamin K deficiency.

• Rifampin may help when bile acid sequestrants are unsuitable but requires regular liver function monitoring.

• Opioid antagonists like naltrexone may reduce itching, but they are not recommended in advanced liver disease.

Nutrition support
Vitamin and mineral supplements, including calcium and vitamin D, may be necessary if PSC interferes with nutrient absorption or weakens bones. Supplements can be taken orally or via infusion.

Treatment for bile duct blockages

• Balloon dilation opens blocked bile ducts using an inflatable balloon inserted through an endoscope.

• Stent placement involves inserting a small plastic tube to keep the duct open. Stents often need replacement.

Treatment for infections
ERCP procedures and repeated or long-term antibiotics may be required to treat or prevent infections caused by bile duct blockages.

Liver transplant
Liver transplantation is the only known cure for PSC. It replaces the diseased liver with a healthy donor liver. Transplant is considered when PSC causes liver failure, severe infections, bile duct blockages, uncontrollable itching, or cancer. Survival rates after transplant are over 90% at one year and about 80% at five years. PSC can occasionally recur even after a successful transplant.

Prognosis
Life expectancy varies, with an average of 10 to 20 years after diagnosis without a transplant. Disease progression is unpredictable; some people remain stable for years, while others develop complications more rapidly.