Alfred L. Baker, M.D.
Division of Gastroenterology & Hepatology
Northwestern Memorial Hospital
Chicago, IL


Qestion 19
Does PBC liver disease (damage) continue to progress while taking the
regimen of Ursodiol. There have been testimonials asserting that enzyme
levels can return to normal after taking the medication. Does liver damage
and progression stop while taking the Ursodiol?

Answer 19
A number of studies have shown that ursodioxicolic acid improves liver chemistry tests in patients with PBC. Other trials demonstrate that this drug can slow the progression of PBC, delaying the time until transplantation or death. Thus, the drug is widely prescribed for PBC, but the effect is to slow disease progression rather than to stop it.


Question 20
Is it possible to have normal liver function tests, and yet have a liver
biopsy that shows damage? If there is liver damage wouldn't this be
showing in the labs?

Answer 20
PBC is occasionally diagnosed in patients with normal liver chemistry tests, and a biopsy in such individuals usually shows evidence of the disease. Such individuals often have cellular infiltrates around the bile ducts, but this is liver damage, albeit mild. Liver chemistry tests can therefore be normal in patients with a liver biopsy that shows PBC.


Question 21
Is there any correlation between depression, a diminished sense of
self-worth, and the development of PBC or autoimmune diseases?

Answer 21
I am not aware of any studies that show that depression or other psychological disturbances predispose individuals to the development of PBC or other autoimmune liver disorders. Depression and other mental disturbances often accompany chronic illnesses, and these can be managed with a variety of medications, support groups, or perhaps even exercise. The patient's physician can often point to ways to cope with the stress of PBC and other chronic illnesses.


Question 22
Is there any correlation between cardiovascular disease (a build-up of
plaque in the vessels), and liver disease?
Is there a raised incidence of cardiovascular disease, heart attacks in PBC
patients.

Answer 22
Increased cholesterol levels are a well-established risk factor for cardiovascular disease in the general population, and patients with PBC commonly have raised cholesterol levels. However, two prospective studies have demonstrated that despite increased levels of blood lipids, patients with PBC appear not to have a significantly increased risk of cardiovascular disease compared with healthy populations with normal serum cholesterol values. PBC patients often have raised HDL levels, which are cardio protective, and cholesterol in PBC patients is often packaged in lipoprotein X. This packaging of cholesterol may protect PBC patients from adverse cardiovascular events. Some of the popular cholesterol lowering agents called statins can be used in patients with PBC. Nonetheless, I ask my patients to focus on well-established risk factors for heart disease including obesity, diabetes, and smoking. I do not usually prescribe statins for PBC patients.


Question 23
If labs point to PBC what age should you not have a liver biopsy? It would
seem to me if someone that is 80 and their child of 50 has PBC that the
risks of the liver biopsy would not be worth it. What are your thoughts?

Answer 23
PBC is now diagnosed commonly in elderly patients. In my judgment a liver biopsy may not be necessary if there is a positive mitochondrial antibody test and a cholestatic liver chemistry profile, that is a raised serum alkaline phosphatase and no more than mild evaluations of the AST and ALT. Many other disorders that might be confused with PBC can be excluded with serologic tests and a liver scan. However, the decision about a liver biopsy in an older patient should depend on the individual situation, including co-morbid conditions, the risks of a biopsy, and the likelihood of prolonged survival.


Question 24
Could explain the differences between PBC and Primary Sclerosing
Cholangitis?

Answer 24
PBC involves injury to small and medium sized bile ducts in the liver whereas PSC usually affects larger bile ducts both inside and outside the liver. Both diseases have a cholestatic liver chemistry profile, that is a raised serum alkaline phophatase with only minimal increases in the AST and ALT. More than 90% of PBC patients have a positive mitochondrial antibody test, a finding that is quite unusual in PSC. Most PBC patients are females whereas about half of PSC patients are female. Patients with PSC commonly have inflammatory bowel disease, whereas this is an unusual accompaniment of PBC. A liver biopsy can often differentiate between the two disorders. On occasion a test to visualize the bile ducts can identify the specific features of PSC. Either ERCP (endoscopic retrograde cholangiopancreatography) done through an endoscope to place a catheter in the lower end of the bile duct to take an x-ray or MRCP (magnetic resonance cholangiopancreatography) done by radiologists using magnetism can be used for this purpose.


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