We wish to thank all the members on our Doctors Panel, for giving us their time answering PBCers questions. We greatly appreciate all they do for us.
Howard Worman, M.D.
Question 1
I've heard varying opinions as to how often a person with PBC should have a biopsy. Is there a "rule" regarding how often this procedure should be performed for diagnostic purposes? Also, are there any situations or extenuating circumstances that would require having a biopsy more often than normally recommended?
Answer 1
There is no rule regarding how often a person with PBC should have a liver biopsy. Most liver specialists would agree at least once to make the diagnosis. Some doctors may suggest a biopsy every 5 to 10 years to determine how the disease is progressing but others would not. This is controversial. Situations that would require a liver biopsy would include a change in condition that could not readily be explained by the natural progress of PBC (e.g. the doctor suspects another liver problem).
Question 2
Are there any new studies or data that would suggest methotrexate is a good treatment for PBC? If the answer is no, are there any circumstances under which you would put a PBC patient on methotrexate (other than being part of a test)? Again if the answer is no, what is the argument for not using methotrexate when some doctors seem to get good results with it.
Answer 2
The following paper was just published this month:
Combes B, et al. Methotrexate (MTX) plus ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis. Hepatology. 2005;42:1184-1193.
Patients were randomized to received methotrexate (MTX) plus ursodiol (UDCA) or UDCA plus placebo. The major conclusions, from the papers abstract, were:
The trial was conducted with a stopping rule, and was stopped early by the National Institutes of Health at the advice of our Data Safety Monitoring Board for reasons of futility. In conclusion, methotrexate when added to UDCA for a median period of 7.6 years had no effect on the course of PBC treated with UDCA alone.
Question 3
What kind of a doctor should one see for Sjogren's Syndrome which often accompanies PBC? Could you explain if Sjogren's is a separate disease or symptom of PBC.
Answer 3
A patient with Sjogren syndrome should see their internist; if patient has PBC, the patient should see the doctor who treats the PBC. Depending on the symptoms, that doctor may refer to another specialist (e.g. an ophthalmologist if eye dryness is a major problem). Sjogren syndrome is a constellation of symptoms that is often associated with PBC but can occur independent of PBC.
Question 4
Is the drug Zetia for cholesterol safe to take with pbc? If not, can you recommend any other drugs for high cholesterol?
Answer 4
Deciding what drug is appropriate for an individual patient can only be determined by a doctor who has obtained a complete history and examined the patient. I am not aware of any data looking a Zetia per se in PBC. It is probably safe but the benefit of reducing serum cholesterol levels in patients with PBC has not been established.
Question 5
I'm under the impression that bile is the culprit in PBC. Please define bile - how is it produced, what exactly does it do or doesn't do.
Answer 5
Bile is a fluid containing water, electrolytes and organic molecules including bile acids, cholesterol, phospholipids and bilirubin that flows through the biliary tract (the ducts that drain the liver) into the small intestine. Bile salts and bilirubin are produced in the liver. In the intestine, the bile salts help with the absorption of fats and fat-soluble vitamins. In PBC, there is damage to the small biles ducts within the liver and the flow of bile within the liver is impeded.
Question 6
Does having PBC make our immune system compromised? If so, what exactly does this mean?
Answer 6
PBC does NOT make your immune system compromised. The term immune compromised is generally used for individuals who have abnormalities with the immune system that prevents it from attacking virus, bacteria or other microorganisms, making them more susceptible to infections.
Examples would be individuals with certain cancers, receiving cancer chemotherapy, with AIDS or with certain other problems.
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