Primary Sclerosing Cholangitis - A Disease That Affects the Bile Ducts of the Liver

Sclerosing cholangitis is a progressive disease that affects the bile ducts which are located both on the inside and outside of the liver. The progression of this disease leads to the bile ducts becoming fully blocked and eventually destroyed which causes liver failure and a liver transplant which is the only known cure for this disease.

However, a liver transplant is not typically performed unless the liver is severely damaged which tends to occur approximately twelve to eighteen years after a diagnosis of primary sclerosing cholangitis is made.

Causes and Symptoms of Sclerosing Cholangitis

While the cause of this disease has not been definitively determined, medical experts believe it may be caused in individual's who are susceptible to this disease when their immune system has reaction to an infection or toxin.

This disease has also been more frequently diagnosed in individuals who have other medical issues including "IBD" or "inflammatory bowel disease" - particularly ulcerative colitis. Primary sclerosing cholangitis is most prevalent in younger males with a diagnosis typically being made between the ages of thirty and sixty years.

This disease has also been known to occur in some patients who have a family history of sclerosing cholangitis. In its beginning stages, primary sclerosing cholangitis may not cause any symptoms that are noticeable and often times is only discovered after a patient has had blood tests that indicate their liver is not functioning properly.

When symptoms do occur with this disease, they may include pain in the abdominal area, fatigue, fever, chills, unexplained weight loss, diarrhea, itchiness, and jaundice which is the yellowing of the skin and whites of the eyes.

When the bile ducts become narrower and scarred, this causes a reduction in the amount of bile salts that are normally secreted that are needed to for the body to absorb a sufficient amount of fats and vitamins including A, D, E, and K that are fat-soluble.

This can lead to complications and other medical conditions that include osteoporosis, bile duct stones and gallstones, an enlargement of the spleen and liver, easy bleeding or bruising, and "steatorrhea" which is greasy and foul smelling stools caused by excessive fat within the feces.

Treatment for Primary Sclerosing Cholangitis

The primary treatment for sclerosing cholangitis is typically to alleviate the symptoms caused by this disease. Many patients experience itching that is uncomfortable and this is typically treated with anti histamine medications and "bile-acid-binding drugs" including cholestyramine such as "Questran" or "Locholest".

Infections also occur frequently in patients with this disease and antibiotics are typically given to treat the current infection as well as prior to other medical procedures that could potentially cause an infection to occur.

Vitamin supplements are also recommended to these patients as the body loses its ability to absorb the proper amount of nutrients and vitamins, in severe cases when a patient is suffering from malnutrition due to sclerosing cholangitis, these vitamins and nutrients may be given intravenously.

Surgical procedures that are sometimes performed on patients who have severe blockage within the bile ducts include balloon dilation where a balloon catheter is inserted and inflatable to expand the ducts and sometimes a stents - plastic tubes - may be inserted in order to keep the bile ducts from closing.

Another surgical procedure that may be done on some patients is a surgical removal of the blockage where the blockage will be removed and portions of the duct that remain will be connected so as to allow the bile to continue to flow through the duct. In those patients where the disease has progressed to the point of causing liver failure, the only treatment that can be done is liver transplantation.