Nathan M. Bass, MD, PhD
Professor of Medicine
Medical Director, UCSF Liver Transplantation Service
Division of Gastroenterology
UCSF Medical Center and School of Medicine
Box 0538, Rm 357-S
San Francisco, CA 94143-0538


QUESTION 1
Is one or two glasses of wine a week harmful in people with Stage 1
of PBC, and taking Urso 4 times a day?

ANSWER 1
I doubt it, but why take unecessary risk if you can avoid it?  The
risk of developing significant liver disease secondary to alcohol is
believed to increase significantly in women who consume more than 7
drinks a week. A glass of wine a day meets this risk threshold.  In a
patient with established liver injury, such as we see in PBC, it is
reasonable to assume that an additional injurious liver toxin could
accelerate the damage and shorten the lifespan of the organ. 
Although there is some debate as to exactly how much alcohol is safe
or unsafe in conditions such as hepatitis C (in which alcohol is
known to accelerate the course of scarring and interfere with medical
treatment), most physicians strongly counsel against drinking any
alcohol in patients with hep C.  The same prudence would seem to
apply to  PBC, although there is much less data.  This probably
reflects the fact that most patients with PBC swear off alcohol
completely. I think this is wise.


QUESTION 2
What can cause weakness and mild confusion that comes and goes? 
Could it be high sed rates (60) or could this be caused by stage 2
PBC?

ANSWER 2
In early PBC, there may be significant fatigue the cause of which is
unknown.  This fatigue can slow patients down, and cause episodic
impaired concentration.  In more advanced disease, the build up of
ammonia and other toxins in the blood stream can cause hepatic
encephalopathy (HE), which is a reversible neurological condition.
Mild HE can cause loss of concentration (driving may become unsafe),
inability to sleep at night and sleepiness during the day.   More
advance HE can cause marked confusion, behavioral changes and even
coma.  In stage two PBC, weakness and fatigue (but probably not  mild
confusion) may be related to the inflammation (and high sed rate)
associated with the disease process.  Even early HE is unlikely at
this early stage of the disease.  Other factors that can contribute
to drowsiness include antihistamines and sedatives used to treat
itching.


QUESTION 3
Many of us suffer terrible itching, and we take URSODEOXYCHOLIC ACID
250mg.  In many it seems to make no difference.  What else can help
with the PBC itching and are there any medications?

ANSWER 3
There is a great deal written about the control of itching in PBC,
but unfortunately, this remains one of the most challenging symptoms
to tolerate and treat.  URSO is really not very successful, but in a
small percentage of patients, it works extremely well, so it is worth
a try.  It affords some relief in up to a quarter of patients. 
Itching is very subjective and individual in its severity. 
Approaches to treatment start with good skin care, including
moisturizers, loose cotton clothing, keeping cool, and avoiding hot
showers and baths (lukewarm is OK).  Keeping nails well trimmed is
important as scratching damages the skin, causing it to dry out and
become even more itchy.  An antihistamine or mild antihistamine-
antidepressant, (e.g., doxepin), especially at  night, is helpful,
but can make one drowsy.  The next line of treatment includes
cholestyramine and ultimately may make use of more potentially
hazardous (but sometimes very effective) drugs such as rifampin and
naltrexone.  Plasmapheresis is a major undertaking and its effects
are usually very short lived.  In the most severe and unrelenting
cases of itching in PBC, early liver transplantation (cadaveric or
living donor) may be indicated, and cures this terrible symptom.


QUESTION 4
Does deep cracking heels and peeling skin on the feet have anything
to do with pbc?  Do you have any idea what can cause this?

ANSWER 4
Dry skin is common in PBC, mainly because of scratching itchy areas,
which may include the soles of the feet.  Dry cracked heels occur in
some individuals in the absence of any systemic disease; indeed this
is a common skin problem and may be helped by a good pedicure, good,
soft shoe orthotic inserts, and treatment of bacterial or fungal
infection that may complicate (or cause) cracked heels.


QUESTION 5
Please offer your opinion on the use of general anesthesia for
elective surgical procedures (non-disease related).  Are  there any
specific risks for a pbc patient?

ANSWER 5
In early PBC (stage 1-2), when liver function is good, the risk of
general anesthesia for elective surgery is not increased.  In
patients with established cirrhosis, there is an incremental risk
that correlates with the degree of failure of liver function.  Thus,
a patient with advanced liver disease will have a high risk of
complications and death from elective, non-liver surgery.  In a
patient with mild liver decompensation, these risks are a lot less,
but not negligible.  Anesthesiologists are generally familiar with
these risks and also what precautions to take in such patients during
surgery.  Some are more experienced than others, however, in dealing
with the administration of anesthesia in patients with significant
liver disease.  PBC patients are not specifically at greater risk
than patients with other types of liver disease, and may actually be
able to deal with anesthesia and the metabolism of administered drugs
better because of well-preserved liver cell function, despite the
presence of cirrhosis.


QUESTION 6
(a) Are liver transplantees who had PBC good candidates for
discontinuance of Prednisone if they remain on Prograf and Cellcept
if no rejection episodes?  (b) Is any test that could predict who is
a good candidate for steroid discontinuance?  (c) If steroids cannot
be safely discontinued, what is the lowest possible dose?

ANSWER 6
(a)  There are no clear answers to this question.  There are
certainly compelling reasons for stopping prednisone after many years
in PBC in order to reduce the risk of bone loss. On the other hand,
there is some data that suggests that patients with "autoimmune"
liver diseases, including PBC, run a higher risk of recurrent disease
if prednisone is discontinued too early.  Recently, a large study
suggested that the risk of recurrent PBC after transplantation was
greater only in patients treated with tacrolimus, which would be the
majority these days.  In patients with bone thinning after
transplant, I try to lower the dose of prednisone or discontinue it
completely within the first 5 years after transplant.


(b) Unfortunately, there is not. This is still determined by trial
and error. But, steroid discontinuation in patients who have had
little or no rejection following transplant seems less problematic.


(c)  Some patients do fine on quite small doses (2-3 mg per day),
despite being unable to tolerate complete steroid discontinuation.
This is achieved through slow, and carefully monitored dose reduction


END


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