Post
transplant
Dr. Howard
Worman
Division of Digestive and Liver
Diseases
Departments of Medicine and of Anatomy and Cell Biology
College of Physicians & Surgeons
Columbia University
New York, NY 10032
Question
25
This may be a radical thought,
or maybe one already considered and dismissed for good reason: Since many
of us have autoimmune diseases other than just PBC, I was wondering if the
drugs used to suppress the immune system after transplant (which probably
decreases the chance of PBC recurring) could be used as therapy years before
transplant in order to control the symptoms of many of our autoimmune diseases.
Answer
Many medical investigators are
considering your thought and it is neither "radical" nor has it been "dismissed
for good reason." Some of the same drugs used to prevent transplant rejection
such as tacrolimus, cyclosporin A and mycophenolate mofetil are being
investigated in the treatment of various autoimmune disorders. More studies
are necessary at this time.
Dr. Howard
Worman
Division of Digestive and Liver
Diseases
Departments of Medicine and of Anatomy and Cell Biology
College of Physicians & Surgeons
Columbia University
New York, NY 10032
April 2000
19. If immunosuppression can help
prevent recurrence of PBC, isn't it plausible that immunosuppression could
help deter the disease if it were provided in the early stage?
Answer
I'm not entirely sure of what you
mean by "prevent the recurrence of PBC." I'll assume that you mean after
liver transplantation. If so, this is not the same situation from an
"immunological point of view" as the natural disease. Proteins on the cells
of the transplanted liver that play important roles in recognition by the
immune system are different. To put this another way, the new liver may not
be susceptible to PBC. In addition, the cellular targets recognized by the
immune system in transplant rejection are probably very different from whatever
targets are recognized in PBC (nobody know what they are in PBC). Therefore,
immunosuppression that keeps allograft rejection in check may not significantly
deter the immune response against the liver in PBC.
Most trials of immunosuppressive
agents to treat PBC have been disappointing. Corticosteroids, azathioprine,
cyclosporin A, chlorambucil and methotrexate have all been tested and no
trial has shown conclusive positive results. Trials of other immunosuppressive
agents are currently in progress.
I should also comment the phrase
"early stage" in reference to PBC. PBC is never really diagnosed until the
person has some liver disease. It’s really not possible at this time
to know who might develop PBC before there is already liver involvement.
Dr. Marshall
Kaplan
Chief,
Division of Gastroenterology
New England Medical Center
Boston, MA
December 1999
13.) Can PBC reoccur after a transplant
and if so, how common is it?
Answer
It is very rare for PBC to recur
after OLT if adequate immunosuppression is used. I have not seen it in any
of the PBC patients who have had transplants at NEMC since 1983. There is
some controversy because researchers at UC Davis and Mayo have considered
recurrence to have occurred in some of their patients who are well, have
normal blood tests and normal liver biopsies but who have a finding on liver
biopsy that is only demonstrable using a research technique,
immunohistochemistry. In my opinion, no one knows what this finding really
means. I would not consider these patients to have recurrent PBC.
Dr. Melissa
Palmer Answers Our Questions
Specialty:
Gastroenterology and Hepatology
Medical advisory board of the ALF
New York Chapter
ALF National Chapter Nutrition
Education Subcommittee
November 1999
1.) I have a question for the doctors,
I have no medical background. I was wondering, with a live donor transplant
the donor gives part of his/her liver to the person in need and the donor's
liver regenerates itself. The transplants liver also takes the new section
and regenerates or grows. What would happen to a PBC liver if part of it
were surgically removed? Would it regenerate on it own, and would it still
have the PBC or would it be disease free? Is this a possibility?
Answer
Living- donor transplantation has
significantly helped the liver-donor shortage especially in the pediatric
are group. It is just recently becoming a more accepted option for adults.
Using this technique, for an adult with PBC, for example, the donor, donates
part of his or her liver to the PBC patient. The PBC patient has his or her
liver totally removed, and the part of the donor's liver put in its place.
It will eventually grow to a normal sized liver. Only "part" of the PBC liver
is not removed, the whole liver is removed.
However, in theory, if part of
a PBC liver is surgically removed, if cirrhosis ( stage 4 PBC) were already
present, the liver could not regenerate. If cirrhosis were not present, the
liver could regenerate, but the newly regenerated liver would still have
PBC.
Dr. Melissa
Palmer Answers Our Questions
Specialty:
Gastroenterology and Hepatology
Medical advisory board of the ALF
New York Chapter
ALF National Chapter Nutrition
Education Subcommittee
November 1999
4.) We know that PBC is an Auto
Immune Disease. If our Immune systems are attacking our liver, why is it
that we think a Liver Transplant would fix our problem doesn't it seem to
reason that the immune system would then attack our new liver? Couldn't this
then be the reason that we have some recurring PBC cases Post Transplant?
Answer
This is a very good question that
has sparked much debate among hepatologists for many years. It does appear
that PBC can recur in the new liver. However, since this disease is so slowly
progressive, it will take many years for any significant damage and/or symptoms
to occur, if they occur at all. Therefore, a liver transplant would certainly
fix the problem for most individuals.
Dr. Howard
Worman
Division of Digestive and Liver
Diseases
Departments of Medicine and of Anatomy and Cell Biology
College of Physicians & Surgeons
Columbia University
New York, NY 10032
April 2000
24. Is Actigall or Urso normally
used in a blocked bile duct after transplant? How successful are stints?
At this time are there any other treatments available?
Answer
Biliary obstruction ("blocked bile
duct") occurs in about 15% to 25% of patients after liver transplantation.
Endoscopically placed stents are sometimes successful in relieving the blockage.
I cannot give a precise answer as to "how successful" stents are. Balloon
dilation of the obstruction is another treatment that sometimes works. Surgical
reconstruction is very often the treatment of choice for bile duct obstruction
after liver transplantation. Ursodiol (Actigall or Urso) is probably of little
or no benefit in large bile duct obstruction that occurs after liver
transplantation.
Dr. Howard
Worman
Division of Digestive and Liver
Diseases
Departments of Medicine and of Anatomy and Cell Biology
College of Physicians & Surgeons
Columbia University
New York, NY 10032
April 2000
Question
42
Other than URSO what is the treatment
to lower indirect bilirubin in a post transplant PBC person?
Answer
There are many causes of elevated
bilirubin concentrations. Elevated bilirubin in the blood per se is NOT something
that is treated, except in infants in whom very high concentrations of bilirubin
can cause problems with the undeveloped nervous system.
If a patient has an elevated serum
bilirubin in blood post-transplant or otherwise, a diagnostic work-up must
be performed to determine the causes. The underlying cause is treated. URSO
(ursodiol) is NOT indicated to "lower the bilirubin" in any condition. It
is used in PBC because in several studies it has been shown to slow the
progression of the disease, not because it lowers the blood bilirubin
concentration.
The Mount Sinai
Medical Center
New York, New York 10029
February 2000
17) If you have diseases such as
gerd, sojgrens, arthritis etc., that seem to be triggered with the onset
of PBC, do they go away or diminish after transplant?
Answer
To the extent that certain diseases
associated with PBC would respond to the immunosuppression used after a
transplant, some symptoms might resolve. For example, if one had rheumatoid
arthritis, prednisone used after the transplant might ameliorate some symptoms.
Gastroesophageal reflux disease (GERD) is not linked to PBC and would probably
not resolve after transplant. I don't think anyone has looked specifically
at Sjogrens syndrome; perhaps we should. I would guess the symptoms would
not change since diseases associated with PBC are just that, associated but
not caused by PBC, and they tend to run their own courses.
Alfred L. Baker, M.D.
Division
of Gastroenterology & Hepatology
Northwestern
Memorial Hospital
Chicago, IL
2000-2001
Question
17
Since everything is processed through
the liver, is there any reason a pre or post transplant PBC patient should
avoid hair color, or nail polish?
Answer
We recommend that all patients
who receive liver transplants avoid hair coloring and permanents for the
first few weeks after the procedure. Otherwise, patients with PBC do not
need to avoid these cosmetic enhancements.
David Bernstein, M.D.
Chief, Division of Gastroenterology
North Shore University Hospital
Manhasset, NY
7/13/2003
Question 2:
For patients who have suffered from severe itching prior to transplant….is
there a percentage that still suffer from itching after transplant?
Answer 2
PBC patients with severe pruritus who undergo transplantation should have
complete resolution of pruritus following the surgery. The mechanism of pruritus
is unclear but it is related to advanced liver disease or the inability to
appropriately process substances which cause itching. Therefore, the new
liver should not be affected. Rarely, itching can occur after transplantation
but this
is usually secondary to the immunosuppressive agents being used, not the
return of PBC.
David Bernstein, M.D.
Chief, Division of Gastroenterology
North Shore University Hospital
Manhasset, NY
7/14/2002
QUESTION 26
Do PBC patients with their overactive immune systems have more rejection
episodes than other liver transplants?
ANSWER
Unlike patients with autoimmune hepatitis, PBC patients do not have more
episodes of rejection than other liver transplant recipients. PBC patients,
in general, do very well after liver transplantation and have excellent 1
and 5 year survival rates.