Nancy Bach, M.D.
Speciality Liver Diseases
The Mount Sinai Medical Center, New York
Monmouth Medical Center, Long Branch, NJ

Question 25
Should there be any restrictions on the diet of one with PBC even in the early stages?  What would you consider the proper diet for those with PBC? 

Could a diet like Weight Watchers be the one (balanced)?  Also, I've read about low protein/high carbs plans or a low fat diet for this
particular disease.  What are your thoughts?

Answer 25
Though much is written and discussed about diet and PBC, research does not reveal a particular diet that is good or bad for the disease itself.  With that said, the importance of staying healthy cannot be underscored enough.  I always recommend that patients follow a low fat, low cholesterol diet.  For those with advanced disease who may suffer complications of the disease, salt and protein restrictions may be necessary.  I happen to love weight watchers. I think it's a balanced diet that is very compatible with real life. 


Question 26
We are told the most important marker for referral to transplantation is the bilirubin.  If this is the case, why are all the regular tests required if the bilirubin is close to the normal range, eg. ultra-sounds, biopsies, cat-scans, etc.  If the bilirubin does increase, at what stage should we be start being concerned?

Answer 26
The bilirubin is one of the most important markers of disease progression.  A rising bilirubin (over 3-4mg/dl and climbing) is a frequent indication for transplant. I don't have a particular protocol for repeat liver biopsies.  The clinical and histologic (liver biopsy) picture of PBC don't always correlate with each other.  My indication for biopsy would either be as part of a research protocol (ex. to see the effects of a particular medication on the disease) or when the clinical picture changes and you need the biopsy to determine what is going on. 
Imaging studies such as CT scans and ultrasounds are also not part of a particular protocol.  In other liver diseases (ex. Hepatitis B and C) we use scans to screen for primary liver cancer.  Because the risk of liver cancer is so low in PBC, screening guidelines do not exist.   With all of that, it is important to defer to the judgment of your own physician.  If a particular study is recommended by your doctor, I think it is always reasonable to ask what they are looking for with the test.  In my practice I like to have an initial biopsy if possible, to confirm the diagnosis, exclude other conditions and to get an idea of the amount of scarring present.  I don't do follow-up liver biopsies unless they are in a study protocol OR if a situation comes up that is unexplained by PBC alone. 



Question 27
Is there any relationship to PBC and Lupus Anticoagulant Antibody?
Will one affect the other or the ability to keep a good INR?

Answer 27
Not much is written on this association.  This antibody is associated with a particular syndrome that may cause an increased tendency to form clots, lower platelet counts and may be associated with an increased rate of fetal loss.  This antibody, like others is often found in individuals with autoimmune diseases including PBC.  The INR should not be affected by the antibody. 


Question 28
Have read a recent article that some pbcers are trying Budesonide along with
urso...is this a good med for us?

Answer 28
Budesonide is a synthetic corticosteroid.  Like prednisone it is an anti-inflammatory and immunosuppressive agent.  The difference between budesonide and other steroids is that budesonide has a high affinity for the liver and much is extracted by the liver and broken down within the liver.  Theorectically, this should decrease some of the adverse effects of taking steroids.  A very small preliminary study, suggested that the combination of budesonide and ursodeoxycholic acid might be better than urso alone, at least in terms of the liver chemistries.  However, another similar study found that a suboptimal benefit occurred from the addition of budesonide.  Additionally, this study found an increase in the risk of fractures, something previously reported with the use of prednisone in PBC.  Unfortunately, like many immunosuppressive agents tested before it, budesonide does not seem to be the answer.


Question 29
What is your opinion of PBCers taking cholesterol lowering drugs, particularly statins?  Which, if any, is the safest to take?  What about Milk Thistle?

Answer 29
Another excellent question for which the data is limited.  We all know that cholesterol levels may be elevated in PBC.  Older data suggested that the elevations were related to the abnormal measuring of different proteins and did not necessarily correlate with an increased risk of heart disease.  With that said, this finding does not make PBCers immune to heart disease.  In addition to that, a more recent study suggested an increased risk of cardiovascular diseases in patients with PBC because of the cholesterol elevations.  With that as background, several groups have reported small numbers of patients with PBC taking statins.  The results of these studies seem to suggest that the drugs are reasonably safe and well tolerated.  We have started to enroll patients in a study to determine the effect of the statins on the course of disease in PBC.  We are hoping that some of the anti-inflammatory and immunosuppressive properties of this class of drug will be beneficial in PBC. Of the statins on the market, I don't believe that one is safer than another.

Milk thistle is probably safe but has never been proven to be of any benefit in PBC.  Because these so called "natural" products are often grown outside of the US and are unregulated, I'm always leery about recommending that patients use such herbs.  One never knows what they are really taking. Without any evidence to suggest a benefit from this plant, I therefore would not recommend it.


Question 30
Am I correct in assuming that those with PBC should not take the smallpox vaccine? What are your thoughts about a PBCer living with others who have had the vaccine?

Answer 30
I have to start with a disclaimer stating that I am not an expert on the small pox vaccine and such a question would have to be referred to the NIH…

With that said, I think that in an individual with well compensated PBC and not taking immunosuppressive medications such as prednisone or methotrexate, the risk from the vaccine would be similar to those without PBC.  If one has advanced liver disease or is taking an immunosuppressive medication, the vaccine would be contraindicated.

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