PRIMARY BILIARY
CIRRHOSIS ORGANIZATION
aka PBCers Organization
We wish to thank all the members on our Doctors Panel, for giving their time
answering PBCers questions. We greatly appreciate all they do for us.
The following questions were answered by:
Henry C. Bodenheimer, Jr., MD
Chief, Division of Digestive Diseases
Beth Israel Medical Center, New York, NY 10003
2004
Question 1
Could you tell me why is it important to avoid infections in PBC.
Should PBCers traveling outside the
Answer 1
The use of shots, or more precisely, vaccinations
before travel is to prevent communicable diseases exposure. There are
multiple vaccinations used to prevent disease, but the use of hepatitis
vaccination is particularly important to people with underlying liver
disease. Hepatitis A and B can be readily prevented with vaccination
and since patients with other chronic liver diseases such as chronic hepatitis
C, primary biliary cirrhosis, or another chronic
liver disease already have an injured liver it is wise to prevent further
injury. Hepatitis vaccines are safe and effective. Vaccinations
do, however, take time to work. Typically, if time allows, the hepatitis
B vaccine is given in three shots over a six-month period. The hepatitis
A vaccine is given once with a booster dose at six months. This time
frame allows the maximum effectiveness of these vaccines. Shorter schedules
are available, as time allows, before travel. Another infection which
travelers should avoid is Vibrio infections.
This type of infection is acquired through raw contaminated shellfish.
People with chronic liver disease should avoid raw clams or oysters.
Question 2
Is ultrasound a useful alternative to biopsies to
detect cirrhosis?
Are most doctors still requiring a liver biopsy to diagnosis PBC and check
progression?
Answer 2
An ultrasound examination is not a sensitive measure
of the amount of fibrosis in the liver. The ultrasound findings of
patients with developing fibrosis are non-specific. Patients with advanced
cirrhosis can be detected by ultrasound, however,
the majority of patients wish to have their disease detected at an earlier
stage. The use of liver biopsy is confirmatory in a diagnosis of primary
biliary cirrhosis. The diagnosis can be made
without biopsy, particularly in the typical female patient with coexisting
autoimmune disorders, such autoimmune thyroid disease in the presence of
mitochondrial antibody, elevated immunoglobulin M, and elevation of liver
blood tests such as alkaline phosphatase.
In patients who are less typical, liver biopsy helps to confirm the
diagnosis. Liver biopsy is also useful in assessing the amount of fibrosis
and often helps to inform patients of their prognosis. The recently
promoted tests measuring the amount of liver fibrosis by blood test have
not been standardized for use in primary biliary
cirrhosis and do not take the place of a liver biopsy.
Question 3
Do people with PBC or other autoimmune diseases have a greater chance of
getting Lymphoma or any other cancer? Do you know what type of affect
would chemo have on PBC?
Answer 3
The issue of an increased incidence of cancer in
patients with primary biliary cirrhosis has been
investigated for many years. Early uncontrolled reports suggested an
increased incidence of cancers such as breast cancer and
lymphoma On closer examination however, with
population controlled studies, the incidence of cancer appears to be similar
to the general population. There is a risk of development of primary
liver cancer in patients with established cirrhosis. This is most commonly
seen in patients with viral hepatitis, but patients with cirrhosis of any
kind may have an increased incidence of primary liver cancer.
Chemotherapy can be toxic to the liver. In patients with advanced liver
disease, particularly those with elevations of
bilirubin the dosage of chemotherapy needs to be
altered and reduced to be safely administered. The action of chemotherapy
balances toxicity against cancer cells with toxicity against healthy
cells. Underlying disease makes the tolerability of chemotherapy more
difficult and patients with impaired liver function have less reserve in
dealing with potential liver toxicity than patients without underlying liver
disease. Chemotherapy is not contraindicated in patients with primary
biliary cirrhosis, but the degree of liver injury
needs to be assessed by the oncologist so that the proper choice of drugs
and their dosage can be prescribed.
Question 4
Do doctors know how long the PBC process has been in the body before elevated
LFTs show up? Could this start in childhood and
not diagnosed until middle age? Could PBC be more common than previously
thought?
Answer 4
Doctors do not know how long PBC has been affecting the body before liver
tests are elevated. However, many patients have been followed with
elevations of sensitive liver markers, such as GGT while they are completely
asymptomatic. Liver biopsies of these patients show very mild non-specific
changes without significant scarring. Although not clearly defined,
patients can have normal alkaline phosphatase levels
with abnormal GGT for months to years before alkaline
phosphatase is elevated.
Bilirubin is a late finding of disease and usually
only seen after decades of disease. There is no indication that this
disease commonly starts in childhood. The trigger of the disease is
not known, but environmental factors (either toxins or viruses), possibly
in combination with underlying genetic susceptibility to the disease are
key features. PBC has been increasingly recognized in the population
and is more common than generally thought. There are a large number
of asymptomatic patients but, I doubt that there is a significant burden
of disease in children.
Question 5
Is weight training safe for those with PBC? I have
heard it could harm veins in the liver. What type of exercises do you recommend
to your patients?
Answer 5
Weight training should be done in moderation. Weight training should
be avoided in patients with true cirrhosis or evidence of portal
hypertension. Patients who have only mild liver disease can participate
in weight training. I recommend that patients pursue aerobic exercise
as their primary activity as a component of weight control and good
cardiovascular health.
Question 6
a. Is there a correlation between PBC and female infertility?
b. Can Urso cause
harm, if taken in the first trimester.
c. Is it safe for PBC patients to take
Clomid for infertility?
d. Do you have any other recommendations for
pregnant PBC patients?
Answer 6
a. Patients with PBC have an increased rate of infertility as do patients
with chronic liver disease in general.
b. There are not good studies of the use of Urso,
or many medications, early in pregnancy. I am not aware of bad outcomes
of pregnancy related to Urso
although, caution is generally advised when
administering medications during pregnancy. Transient discontinuation
of Urso during pregnancy should not have long-term
adverse effects in the treatment of PBC.
c. Hormonal infertility treatment can
be associated with hepatotoxicity and causes a
greater risk for those patients with known liver disease, particularly
cholestatic liver diseases such as primary
biliary cirrhosis and is generally ill advised.
d. In patients who have become pregnant and
who have chronic liver disease, I have advised assessing patients for the
presence of portal hypertension and esophageal
varices. The presence of esophageal
varices should be determined before delivery so
that obstetricians and the patient's physicians may be prepared for potential
complications. In patients with well compensated liver disease pregnancy
may continue normally and no specific alteration of pregnancy care
nor delivery need to be made. Patients should
have adequate vitamin K administration if
prothrombin time is prolonged or if the use
of cholestyramine
has induced blood clotting abnormalities.