Primary Sclerosing Cholangitis (PSC) is a disorder in which the bile ducts undergo inflammation and scarring. The blockage in the bile ducts leads to the accumulation of bile fluid in the liver and secondary damage to liver cells. Additionally, the lack of proper release of bile fluid into the digestive system reduces the body's ability to digest various foods and absorb different vitamins. The cause of PSC is unknown, but there is a hypothesis that the disorder might result from an autoimmune process where the body's immune system attacks itself. In many cases, PSC is associated with inflammatory bowel disease, particularly involving the large intestine.
Primary Sclerosing Cholangitis progresses very slowly, and it can take many years from the onset of the disease until its diagnosis. Due to the blockage of the bile ducts, bile fluid is not properly drained, which can lead to jaundice and itching. Furthermore, inadequate release of bile fluid can result in poor absorption of fat-soluble vitamins (A, D, E, and K). Bile stagnation in the blocked ducts can become a source of infections and a condition known as "ascending cholangitis," which can cause fever, chills, and systemic infection. Over time, the liver can be damaged by the accumulated bile, leading to liver failure.
Suspicion of Primary Sclerosing Cholangitis is occasionally raised incidentally with abnormal blood test results that require further investigation. The diagnosis is usually performed using a specialized MRI called MRCP, which can detect subtle changes in the bile ducts both within and outside the liver. Sometimes, a procedure similar to an endoscopy called ERCP or a liver biopsy may be needed to confirm the diagnosis and assess the extent of damage.
While there is no cure for Primary Sclerosing Cholangitis, we can provide supportive treatment to manage symptoms and complications of the disease. Antibiotic devices may sometimes be needed temporarily or even regularly for treating infections if PSC occurs frequently. Endoscopic procedures can be performed to widen the bile ducts and alleviate blockage in selected cases, along with adding medications to control itching. If the disease progresses to liver failure, liver transplantation might be considered, as outcomes after transplantation are generally very good.