Cholestatic Diseases

A cholestatic disease is one that leads to cholestasis, a condition where the flow of bile from the liver is blocked. (Bile is a fluid produced by the liver that helps digest fats and performs other important bodily functions.) This can lead to cirrhosis—or scarring—of the liver. Two of the most common cholestatic diseases are:

Primary Biliary Cirrhosis 

Primary biliary cirrhosis (PBC) refers to a condition in which the bile ducts in the liver become swollen (inflamed) and are slowly destroyed, leading to cholestasis and often cirrhosis. Its cause is not definitively known, although it is often considered an autoimmune disease (a disease that leads the body turns against its own healthy cells). Approximately 90 percent of patients with PBC are female.

Many patients diagnosed with PBC remain symptom-free in the early stages of the disease, although some experience fatigue and itchiness, among other symptoms. As the disease progresses to its later stages, patients can develop symptoms associated with a damaged liver such as jaundice and digestive problems. At this point, a number of serious problems can occur. The most prominent of these is extensive cirrhosis, which impairs the liver’s ability to function properly in a number of ways and can ultimately lead to complete liver failure.

Treatment for Primary Biliary Cirrhosis

While there is no cure for PBC, treatment typically includes medications meant to slow the progression of the disease. Once the damage becomes too significant for the liver to function properly, transplantation may be the best option for the patient. PBC patients often do very well following transplant, although the condition may recur in the new liver.

Primary Sclerosing Cholangitis 

Primary sclerosing cholangitis (PSC), like PBC, is a chronic condition that results in damage to the bile ducts in the liver. Unlike PBC, however, PSC also leads to damage of bile ducts that are located outside the liver. As the bile ducts cease to function properly, cholestasis develops, ultimately leading to cirrhosis of the liver. PSC is considered an autoimmune disease and it predominantly affects males. Often, patients with PSC also have an inflammatory disease of the colon known as ulcerative colitis. The symptoms of PSC are similar to those of PBC, and many patients with PSC have no symptoms in the early stages of the disease.

Treatment for Primary Sclerosing Cholangitis

PSC progresses slowly, but it typically ends with liver failure and the need for a liver transplant to prolong life. Treatment for those diagnosed with the disease focuses on reducing the symptoms of the disease as it advances. Unlike with PBC, no medications have been shown to slow or reverse the progression of liver damage caused by PSC. This means that liver transplant is the only cure for the condition, although the procedure is typically reserved for those patients who have reached liver failure or have developed other serious complications of the disease.

We Can Help

For additional assistance from an outreach liaison, please contact:

Sharyn Kreitzer, MSW
Senior Outreach Liaison

Transplant Living Center

With amenities including ten bedrooms and two kitchens, the Transplant Living Center (with concierge Allyson Reala) is a "home away from home" for patients and families.