Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic autoimmune disease with inflammation and stricture formation in the bile ducts inside the liver and/or in the extrahepatic bile duct which drains into the small bowel (CBD). The cause is unknown. PSC is closely linked with autoimmune colitis like ulcerative colitis or Crohn’s colitis. Potential complications of bile duct scarring include bile duct infections (cholangitis), cirrhosis of the liver, and bile duct cancer.

Often patients have no symptoms but abnormal liver chemistries are detected with blood check up. Patients can present with fatigue, itching, jaundice or fevers. There is no specific serology test for PSC. Diagnosis is suggested by abnormal imaging tests such as ultrasounds or magnetic resonance imaging (MRI). Liver biopsy is required in some cases.

PSC and Colitis

Patients with PSC often have colitis without any symptoms. Every patient should have a colonoscopy and random colon biopsies. There is increased risk of colon cancer in PSC patients, and patients need frequent colonoscopy examination to detect early bowel cancers. It is recommended on an annual basis.

PSC and Biliary Cancer

There is an increased risk of bile duct cancer in patients with PSC. Because of this, screening is recommended every 6 to 12 months with ultrasound or MRI. Smoking and alcohol increase cancer risk.

Medical Therapy

Medication therapy is available with ursodeoxycholic acid which helps to improve the condition but does not cure. Antibiotics are required often if there is fever and patients may need endoscopic retrograde cholangiography (ERCP).

ERCP involves upper endoscopy with a specialized endoscope through the mouth, dye injection into the common bile duct, and possible dilation or stent placement for severe strictures. Brushing and/or biopsy is often done at the same time to evaluate for bile duct cancers. Small stones in the bile duct can be removed. Although the scope is sterilized, the procedure can be complicated by sepsis with hospitalization. Dilation and stenting improve jaundice and fevers and enable better antibiotic treatment.

Surgery: Liver Transplantation

If there is a recurrent infection, and liver cirrhosis with liver failure develops, liver transplantation offers effective treatment. Liver transplantation allows complete remission of PSC. Decisions about surgery and liver transplant require close coordination between Gastroenterology, Hepatology and the Liver Transplant Team. For this reason, care for PSC is best in major academic medical centers, such as Baylor St. Luke’s Medical Center, which offers liver transplantation. Close communication between the specialty services improves outcomes and patient quality of life.