WHAT IS PSC ?
Primary Sclerosing Cholangitis (PSC) is an uncommon chronic liver disease where the bile ducts inside and outside the liver progressively narrow due to inflammation and scarring. Bile ducts are tubes which carry bile (a greenish yellow liquid made by the liver) into the upper part of the bowel, in order to break up the fat from the food that we eat. The narrowing causes bile to accumulate in the liver, and can result in damage to the liver cells. There is currently no evidence that the disease may be inherited.
PSC may occur alone, but in 70% of cases there is associated bowel disease, particularly Colitis and/or Crohn’s Disease.
WHAT ARE THE SYMPTOMS ?
Initially, individuals may not show any symptoms, and the disease is detected due to abnormal blood test results. Common early symptoms are tiredness, and discomfort in the right upper abdomen. Later symptoms include itching and jaundice. Episodes of chills and fevers from superimposed infection in the bile ducts occasionally occur, and can be distressing.
HOW CAN IT BE TREATED ?
Currently, there is no specific treatment for Primary Sclerosing Cholangitis. Research is underway, but there is little evidence that drugs can reverse existing scar tissue in the bile ducts. Ursodeoxycholic Acid (Urso, UDCA, Destolit) is used to improve bile flow, and this usually improves liver blood tests. Urso may have other therapeutic effects. However, it is still unclear as to whether this has any effect on the progression of the disease. Where patients have isolated strictures, or narrowing in the bile ducts, treatment may involve inserting a stent, which is a drainage tube, through the opening to improve the flow of bile out of the body.
In cases where PSC leads to deterioration of the liver, patients would be considered for liver transplantation. However, because 15 to 20% of transplant patients have serious problems after transplant, this treatment is reserved for patients who have had serious liver deterioration.
WHAT ARE THE ASSOCIATED CONDITIONS ?
As mentioned previously, Colitis and Crohn’s Disease are associated with PSC. The frequent occurrence of PSC in association with inflammatory bowel diseases suggests that a common cause for both diseases may exist, or that the inflamed colon allows toxins or infections to be absorbed into the body, and this causes bile duct inflammation. A small percentage of those with PSC will develop cholangiocarcinoma, which is cancer of the bile duct.