Primary sclerosing cholangitis is a medical condition affecting bile ducts of the liver. Bile ducts get inflamed and as a result there is hardening and scarring of these structures.
Liver Cirrhosis and Liver Failure
Primary sclerosing cholangitis is progressive disease and due to severe damage to bile ducts the person eventually develops liver cirrhosis which subsequently leads to liver failure. The only way to help patients in terminal stage of the disease is to provide a liver transplant.
Unfortunately, there is no cure for the disease. The goal of current treatment is to slow down and reverse damage induced by inflammation. So treatment is basically symptomatic and it also prevents potential complications.
Primary Sclerosing Cholangitis Clinical Characteristics
Patients suffering from primary sclerosing cholangitis typically complain about abdominal pain of different intensity, fatigue, sometimes chills and fever. They also have to deal with frequent bouts of diarrhea. There is evident weight loss which accompanied lack of appetite. Jaundice points to damage to the liver and accumulation of bile under the skin are accompanied by yellowing of the skin and itchiness. As it is the case with jaundice induced by other causes there is also yellowing of the whites of the eyes.
In the initial stage of the disease there are no symptoms and signs and only by routine examination and test doctors may identify inadequate liver function and during further search for the underlying cause confirm the disease.
Primary Sclerosing Cholangitis Causes
Scientists have not managed to identify the actual cause of sclerosing cholangitis yet. However, it is believed that the trigger may be some infection or exposure to a toxin both of which may induce an abnormal immune system reaction and initiate inflammation of bile ducts. The very condition is also reported in people who are already suffering from certain illnesses like inflammatory bowel disease.
Primary Sclerosing Cholangitis End Stage and Complications
Complications of the disease are most commonly seen when the disease has significantly progressed and they are characteristic for end stage of primary sclerosing cholangitis.
Liver disease and liver failure are inevitable complications which develop after certain period of time. Scarring of the liver becomes severe enough and the organ loses its ability to regenerate. As a result patients end up with liver failure. The only way to survive is to undergo liver transplantation. Portal hypertension is a complication closely associated with liver failure. It is a serious medical issue that leads to esopharingeal varices. These may easily bleed and cause even more problems.
Repeated infections in the bile ducts are another complication of the disease. Furthermore, such patients are at higher risk for osteoporosis due to lack of calcium ad vitamin D.
Finally, primary sclerosing cholangitis increases chances for one to develop two types of cancer, bile duct cancer and colon cancer.