Dr Howard Woman Answers The PBCers
Howard Worman, M.D.
Division of Digestive and Liver Diseases
Departments of Medicine and of Anatomy and Cell Biology
College of Physicians & Surgeons
Columbia University
New York, NY
http://www.cc.columbia.edu/~hjw14/index.html
Question 1
Could you please explain the difference between scarring and cirrhosis??
I
thought they were the same and is there a time span from one stage to the
next stage.
Answer
The medical term for "scarring" is fibrosis. Cirrhosis is characterized
anatomically by widespread nodules in the liver combined with fibrosis. The
fibrosis and nodule formation causes distortion of the normal liver architecture,
which interferes with
blood flow through the liver and can also lead to an inability of the liver
to perform its biochemical functions. Fibrosis can be present without nodule
formation. In some cases, fibrosis will not progress to cirrhosis. In other
instances, it can progress in a time span of a few years.
Question 2
I am Stage 2 on Actigall & either Methytrexate or placebo & am in
clinical Trials at MCV. Now they want to try Rifampin - as a nurse practitioner,
I know the reasons for general prescribing and the side effects, etc. but
do not know why this can help with PBC itching. Comments? Any other
suggestions?
Answer
Itching secondary to liver diseases, including primary biliary cirrhosis,
is a very difficult symptom for patients to endure and for physicians to
manage. The reason why patients with liver disease itch is not known. It
has been thought that some substances accumulate in the blood as a result
of liver disease, causing itch. It is not know how many of the drugs that
may help itching in some cases actually work. At our own center
(Columbia-Presbyterian), we have several clinical trials underway to figure
out why some people with liver diseases itch and what drugs may help.
For more information on itching liver liver diseases, I refer the readers
to
the following website:
http://cpmcnet.columbia.edu/dept/gi/itching.html
Question 3
Can you please advise the risks of pregnancy for a patient with PBC?
Is this something we should avoid?
Answer
The risk varies from one case to another. In GENERAL, pregnancy is an increased
risk in patients with cirrhosis. In patients with liver disease without
cirrhosis, pregnancy is generally not a huge risk. Patients with PBC who
are considering getting pregnant should discuss the issue with a doctor who
knows their complete history.
Question 4
Could you please explain in layman's terms, the difference between Autoimmune
Cholangitis and Primary Biliary Cirrhosis? Is there a conclusive test for
AIC, and would it show up in a biopsy?
Answer
"Autoimmune cholangitis" is not a well-defined disease or condition. It is
a very confusing term and not all experts agree as to what it really means
or if it really exists. Some people use "autoimmune cholangitis" to refer
to what has also been called "AMA-negative PBC." This is the group of patients
who have most of the features of PBC but do not have detectable antimitochondrial
antibodies in their
blood. I personally disapprove of these classifications ("autoimmune cholangitis"
and "AMA-negative PBC") as recent data using more sensitive tests show that
many of these patients also have antimitochondrial antibodies. Some reports
suggest that patients with so-called "autoimmune cholangitis" have features
more commonly
seen in autoimmune hepatitis. Some doctors refer to this as "overlap syndrome."
I'm also not really sure that this is a specific disease or condition. In
conclusion, its not really clear what "autoimmune cholangitis" is. Perhaps
it is PBC with some different features.
Perhaps its a separate disease. Until we know what causes bile duct damage
in PBC and similar conditions, nobody will know. More basic research is
necessary! There certainly is no "conclusive test for AIC." And there are
no definitive findings on biopsy either. Even in most cases PBC, the biopsy
finding are usually only "consistent with" the disease and not absolutely
diagnostic.
Question 5
I am confused about the benefits/purpose of taking Actigal/Urso - some doctors
say it doesn't do anything - others that it slows down the progression of
PBC - others that it "improves the numbers" but that doesn't really mean
anything.
What are your comments on taking Actigall/Urso? If it slows down progression
of PBC-by how much? Do you have an idea of percentage of people it slows
progression? If your numbers (ALT-AST) improve -- what does that mean?
Answer
I can't answer this question briefly. Doctors and experts on PBC will debate
these issues for days. In short, several studies do show that ursodiol (the
active ingredient in Urso and Actigall) slows the progression to cirrhosis
and decreases the number of patients who undergo liver transplantation during
several years of follow-up. So
yes, there are some data showing that ursodiol may "slow down the progression
of PBC." It is unclear "by how much" or "in what percentage of people" as
the studies no studies have been designed to answer these questions. It is
not clear what it means if "your numbers improve." There are no data available
to answer this
question. My personal feeling is that the numbers you are referring to (ALT,
AST, alkaline phosphatase) do not mean anything in the long term outcome.
Question 6
Do you see a lot of patients with kidney and bladder infections?
Are they common with people who have PBC?
Answer
Several studies suggest that urinary tract infections are more common in
women with PBC. Our own study (Parikh-Patel A., Gold, E. B., Worman, H.,
Krivy, K. E. and Gershwin, M. E.. Risk factors for primary biliary cirrhosis
in a cohort of patients from the United States. Hepatology. 2001;33:16-21)
showed an increase of vaginal or
urinary tract infections in women with PBC of approximately 4-fold compared
to controls. Some investigators have even hypothesized that having a urinary
tract infection may be a "trigger" for PBC in certain susceptible individuals.
This hypothesis has not been proven.
***If you would like to send questions to our Doctors Panel, please send
your
question to DrPanel@pbcers.org
A doctor cannot be specified. The questions sent in by our PBCers are added
to a list and the next 6 questions on the list are sent to the next doctor
to receive questions. Please make sure the questions you send in are
GENERIC. The doctors will NOT answer questions that are individual specific.
They cannot treat us online.
If you sent in a question and it has not been answered by one of our doctors,
most likely it was considered to individual specific, or was lost in cyberspace.
Please resend it.
HAVE A GOOD DAY.......