Patients with PSC are at risk for developing other associated problems.
A dominant stricture is a blockage in one of the large bile ducts that serve to drain bile from the smaller bile ducts within the liver and deliver it into the small intestine. A dominant stricture occurs in seven to twenty percent of patients with PSC. When a dominant stricture is present, bile is blocked from flowing through the bile duct and, therefore, may back up in the liver. Substances that are normally excreted into bile accumulate in the bloodstream and body tissues. This can lead to jaundice (yellowish discoloration of the eyes and skin), stone formation in the bile ducts and infection in the bile ducts known as cholangitis. At ERCP a piece of plastic tubing called a “stent”can be placed across a dominant stricture in an effort to relieve the blockage.
- Fat Soluble Vitamin Deficiencies
Vitamins A, D, E and K, are known as fat-soluble vitamins because they dissolve in oils rather than water. One function of bile is that it improves the intestine’s ability to dissolve and absorb these vitamins. If bile from the liver does not get into the intestines in adequate amounts, fat-soluble vitamins may not get absorbed and deficiencies can occur. Blockage of bile ducts may also cause diarrhea because fat in the diet will be poorly absorbed.
Osteoporosis or bone thinning is another complication of PSC. The cause of osteoporosis in PSC is unclear. As a result, a bone density scan (also called a dual-energy x-ray absortiometry, or DEXA scan) should be obtained at regular intervals.
The risk of developing cancer involving the bile ducts (known as cholangiocarcinoma) is higher in patients with PSC than in a healthy population. Cholangiocarcinoma may occur in 7 to 15% of patients with PSC. Patients with a long history of inflammatory bowel disease and smokers are at the highest risk. As there are no early symptoms of cholangiocarcinoma that make it easy to distinguish from symptoms of PSC, it is difficult to diagnose early. Researchers and physicians continue to work on ways of making early diagnosis of cholangiocarcinoma.
Cirrhosis is a process of advanced scarring of the liver. Fibrosis or scarring of the liver seen in cirrhosis leads to a decrease of blood flow through the liver. This prevents the liver from performing its critical functions of purifying the blood and nutrients absorbed from the intestines. The end result is liver failure.
Cirrhosis is the seventh leading cause of death in the United States and can be caused by many other processes such as a genetic disorder called hemochromatosis, viral infections, such as hepatitis or by alcoholism. To learn more about cirrhosis and its potential complications, please click here to access a patient brochure published by the ACG on cirrhosis.
Patients with PSC may develop secondary problems that may ultimately result in irreversible liver failure. These problems include bleeding from swollen veins (varices) most commonly in the esophagus, collection of fluid in the abdomen (ascites) and legs, and episodes of confusion (hepatic encephalopathy) due to the inability of the scarred liver to remove toxins from the blood stream.
There is an increased risk of colon cancer in patients with PSC that also have inflammatory bowel disease. Colonoscopy with biopsies throughout the colon once a year is recommended.
What are the treatments for PSC?
Many medications have been studied but none have proven to alter the course of PSC. This is primarily due to uncertainty regarding its cause. Medications such as ursodeoxycholic acid (UDCA), colchicine, steroids and methotrexate have been used to treat PSC. Studies evaluating higher doses of UDCA than had been used previously are ongoing.
Therapy is directed towards treating symptoms, preventing and managing complications of PSC. Patients with PSC should avoid anything that may cause or worsen liver damage such as alcohol. Vaccinations for hepatitis A and B should be administered. Vitamin supplements should be prescribed for patients with advanced disease at risk for deficiencies. Medications such as diphenhydramine, cholestyramine, rifampin and phenobarbital have been helpful in relieving itching. Osteoporosis is managed with supplemental calcium and vitamin D. Treatment of itching and osteoporosis in PSC is similar to that used for treating Primary Biliary Cirrhosis (see below). Alendronate is a medication often used to decrease further bone loss that may help in PSC.
When should liver transplantation be considered?
Patients who develop complications of cirrhosis and those with recurrent cholangitis are candidates for liver transplantation. Liver transplantation is the only effective treatment for patients who have developed late stage PSC (cirrhosis). Studies have shown that approximately eighty to ninety percent of patients who undergo liver transplantation for late stage PSC are alive one to three years later. After transplantation, many patients report having a good quality of life.
Author(s) and Publication Date(s)
Young-Mee Lee, MD, and Marvin Jose Lopez, MD, New England Medical Center, Boston, MA – Published December 2004. Updated January 2010.
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