Primary Sclerosing Cholangitis
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Primary sclerosing cholangitis is primarily a disease of the bile ducts of the liver. It normally affects the bile ducts outside the liver but can also affect the bile ducts inside the liver, as well as those of the gallbladder and pancreas. When inflammation present in this disease causes these ducts to scar and harden, they become narrowed or blocked. Bile cannot drain properly and it accumulates in the liver, causing damage to cells there.
"Cholangitis" refers to the inflammation in the bile ducts that causes the "sclerosing" or scarring process. "Primary" refers to the process not being a result of some other disease. Most patients with sclerosing cholangitis will undergo permanent liver damage in the form of cirrhosis. These patients are at risk for liver failure and for malignant cancer of the bile duct.
This disease is also referred to as "idiopathic" sclerosing cholangitis, meaning that the cause is unknown. It is believed that some abnormal immune reaction directed at the tissue of the bile ducts begins the process.
The presence of inflammatory bowel disease associated with primary sclerosing cholangitis, as is the presence of certain other sclerosing diseases and AIDS. Men are also more likely to have this condition.
Signs & symptoms
The disease may take years to produce symptoms but once they begin to develop, patients experience:
- loss of appetite;
- weight loss;
- and jaundice.
Sclerosing cholangitis brings on nutritional deficiencies and can also cause diffuse itching, abdominal pain, and infection. The disease's course is slow and progressive.
In addition to taking a medical history, performing a physical examination, and requesting blood tests, physicians may order cholangiography to diagnose primary sclerosing cholangitis. In this procedure, a specialist injects dye into the bile ducts to give more contrast definition to x-rays of these ducts. It may be performed by ERCP or PTC.
As the disease progresses, patients may need a liver biopsy, to determine the degree of damage to the liver. A specialist will use a fine needle to remove a small amount of the liver for microscopic analysis.
Treating primary sclerosing cholangitis is a challenge. Care is focused on the symptoms - rather than the underlying process, for which there is no treatment. Drug therapy can address itching and infection. Vitamin therapy can address bone loss.
Specialists may attempt to open obstructed bile ducts by dilation (stretching them open) with balloon catheters or by stenting them. Sometimes surgery to bypass the duct blockage is performed. Surgeons in Aria's Division of General Surgery are experts in providing surgery for conditions of the gallbladder.
The only cure for the condition is liver transplantation, and sclerosing cholangitis is, in fact, one of the primary reasons for which patients will undergo liver transplantation. Gastroenterologists at Aria Health ths work in cooperation with Jefferson's Liver Transplant Program to provide patients with advanced liver disease the opportunity for evaluation and possible transplantation. Survival rates and quality of life after liver transplantation for sclerosing cholangitis are very good.