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Dr. Mason Answers Our Questions

Questions & Answers

May 2000

1.) Any ideas what causes Primary Biliary Cirrhosis? Since 95% are women

diagnosed would it be safe to say the cause is hormonal? Do you know of any

current PBC studies being down other than Dr. Gershwin's at UC ?

Answers

We have been working on the idea that an infection causes PBC. Researchers in

my laboratory have been cloning and characterizing a novel human virus that

may be associated with PBC. In collaboration with Dr. Neuberger in

Birmingham, UK, we have found that normal biliary epithelial cells can be

transformed to appear like PBC diseased cells in the laboratory by mixing

them with the presumed infectious agent from PBC patients. We hope to publish

more details about the relationship between the virus and PBC soon.

There are obviously many other differences between the sexes apart from

hormones, but we believe that they may play a pivotal role in PBC. For

instance, we think that our virus may be stimulated by female hormones but we

have not formally proven this yet.

2.) In your oppinion is it safe to take adult vitamins when diagnoed with PBC

or any other autoimmune liver disease? Instead of taking adult vitamins would

it better for PBCers to take children's vitamins?

Answer

I usually recommend that patients with autoimmune liver disease and biliary

disorders take regular multivitamins with the recommended dosage of any

brand. Patients with biliary disease and those taking oral corticosteroid

treatments are prone to bone disease. Therefore, patients should ensure that

the multivitamin tablet has vitamin D in it and take additional calcium

supplements such as Tums or Oscal 1 to 1.5g per day.

3.) Can you recommned or suggest other treatments for PBC besides Actigall &

Urso medications?

Answer

This is the only recommended treatment for PBC at present but several groups

are trying alternative immune based and other therapies. We have conducted a

pilot study using anti-viral treatment for PBC patients and found that the

treatment was well tolerated. Although no one had a complete biochemical

response, several patients with early disease had marked improvements in

their liver biopsies after a year’s treatment. We will soon be commencing a

second pilot study to assess efficacy and safety of a more potent anti-viral

regimen for PBC patients.

4.) All my life I always recovered quickly from a flu or virus. Since I was

diagnosed with PBC 3 years ago, it now takes weeks to recover from any type

of illness (virus, flu, sinus infection), and I catch anything I come in

contact with. Is this due to the PBC or in general the liver not functioning

correctly?

Answer

The difficulty overcoming infectious disease is probably related to fatigue

associated with chronic liver disease. However, PBC can be related to other

disorders as well, such as thyroid and other autoimmune diseases. So, if you

have any other specific problems, it is worth discussing these with your

doctor.

5.) Do you know of any alternative methods used to help improve the liver and

PBC such as Milk Thistle?

Answer

I usually tell patients that there are no good studies to indicate

prescription of alternative medicines. Milk Thistle appears to be well

tolerated but of unknown benefit. I am not aware of any other good

alternative treatments but I always recommend a daily multivitamin with

vitamin D as well as calcium supplements.

6.) What causes the decrease of vitamin D in a person with PBC? Shouldn't

this be easily corrected by taking vitamin D orally?

Answer

Vitamin D is a fat soluble vitamin that can be inadequately absorbed in

patients with biliary disease. This can be easily corrected by taking 400 IU

vitamin D, which is the usual dose in a standard multivitamin tablet.

August 2000

Question 7

What is the correlation of labs, biopsy and symptoms? Isn't it true a person

can have normal labs and the disease still progress?

Answer

The correlation of labs, biopsy and clinical symptoms is not absolute. The

liver function tests and clinical symptoms can vary with episodes of

deterioration as well as improvement. Even though the stage of the biopsy

progresses from 1 through 4, the level of inflammation can also vary with

progression of the disease.

For all patients we use a combination of liver function tests, liver biopsy

and clinical symptoms to get a general idea of how severe the liver disease

is. Unfortunately you are correct in thinking that a patient can have

cirrhosis when the liver function tests appear to be relatively benign.

However, it is also true to say that the bilirubin becomes raised with

progressive liver disease when patients develop cirrhosis.

Question 8

A metaanlysis published in the Lancet, Volume 354, Page 1053, 25th September

1999, " Randomised controlled trials of ursodeoxycholic acid therapy for

primary biliary cirrhoses, concludes that using UDCA as a standard therapy

has to be checked again as the trials did prove a lack of effectiveness. What

is your opinion?

Answer

Most Hepatologists believe that UDCA has some benefit for PBC patients but it

is no panacea and will not cure the disease. The Lancet Meta-analysis merely

confirmed what most physicians already know. It is clear that UDCA can

contribute to an improvement in liver biopsy and liver function tests.

However, it may not impact that much on delaying the onset of liver failure.

I do not think that we will have to perform any further studies on UDCA as a

single agent as we have a good idea about its utility. However, there are

ongoing studies to assess UDCA as a combination therapy with other drugs such

as methotrexate, I personally think that UDCA will be a good adjunct therapy

with anti-viral treatment.

Question 9

Is it " normal " for Actigall/URSO to reduce the anti-mitochondrial antibodies?

Answer

We still do not know the precise role of anti-mitochondrial antibodies in

PBC. At this time, they are considered a specific marker for ...ETC. >>>>>