Primary Sclerosing Cholangitis
Primary sclerosing cholangitis (PSC) is a chronic, progressive disease in which the bile ducts—both inside and outside the liver—become inflamed, scarred, and narrowed. Over time, these strictures interfere with the flow of bile, which can lead to liver damage, repeated infections, and eventually cirrhosis. PSC is strongly associated with inflammatory bowel disease, especially ulcerative colitis, and may remain silent until complications develop.
What is it?
Primary sclerosing cholangitis causes chronic inflammation and scarring of the bile ducts, which carry bile from the liver to the small intestine. As the ducts become narrowed or blocked, bile cannot flow normally, leading to liver injury. The exact cause is unknown, but PSC is thought to involve autoimmune factors, genetics, and environmental triggers. Most patients also have inflammatory bowel disease, especially ulcerative colitis.
Imaging—particularly MRI with MRCP—shows characteristic findings such as alternating narrowed and dilated bile ducts (“beading”), strictures, and irregular bile ducts. Over time, PSC can progress to cirrhosis, portal hypertension, or bile duct cancer (cholangiocarcinoma), making early and regular evaluation important.
Important to Know
PSC is a long-term progressive disease, and many individuals may not notice symptoms until liver function becomes impaired or complications arise. Because PSC increases the risk of bile duct cancer, gallbladder cancer, and colon cancer (in those with ulcerative colitis), regular monitoring is essential. The condition can also lead to repeated infections of the bile ducts (cholangitis), which may present with fever and sudden worsening symptoms.
Red flag symptoms include fever, chills, new or worsening jaundice, severe itching, confusion, abdominal swelling, or dark urine. These may indicate infection or liver decompensation and require urgent evaluation. Long-term management includes regular imaging, blood tests, and colonoscopy in those with associated inflammatory bowel disease.