PRIMARY BILIARY CIRRHOSIS ORGANIZATION  aka PBCers Organization

We wish to thank all the members on our Doctors Panel, for giving us their time answering PBCers questions.  We greatly appreciate all they do for us.

The following questions were answered by: 
Nancy Bach, M.D.                    
Specialty Liver Diseases  
The Mount Sinai Medical Center
New York, New York

2004

QUESTION 1
Is it possible to have cholestatic LFTs, neg. AMA , a negative biopsy, a mother with PBC and not go on to develop PBC or like conditions with bile duct damage?  Please explain. 

ANSWER 1
This is an interesting question.  I'm not sure what a negative biopsy means.  Had your AMA been positive and the biopsy read as within normal limits, I would have said, you were likely to progress to PBC.   If your AMA is negative and you have abnormal liver chemistries with an unremarkable biopsy, even though your mom has PBC, statistically you are more likely to have an alternate explanation for the abnormal liver biochemistries.


QUESTION 2
Can people have Autoimmune Cholangiopathy and never really progress?

ANSWER 2
Autoimmune cholangiopathy is a questionable term.  I'm assuming you mean AMA negative PBC which tends to behave similarly to AMA positive PBC.  Most individuals with PBC will have SLOWLY progressive disease.  Because the disease is so slowly progressive and there are few ways to really follow for disease progression, for some the thought that the disease never really progresses may be correct.


QUESTION 3
How should I proceed to be proactive in my approach to PBC diagnosis and treatment?

ANSWER 3
Find a doctor who is well versed in the disease.  Be careful about what you read in the library or on the internet.  Much of that data is either skewed or out of date.  The key to living with PBC is to maintain a healthy lifestyle and to enjoy the good quality of life most individuals with this disease have.


QUESTION 4
How do I know if my fatigue is PBC-related or just "normal?"  I feel tired much of the time.  Why is fatigue so common in PBC, and not the same in other liver diseases?

QUESTION 4
Sorry to say, unless you move to Tahiti and remove all outside stresses and still feel fatigue, there is no way to differentiate PBC related fatigue from just "normal" fatigue.  The cause of fatigue in PBC is not totally understood.  Significant data suggests that it is a central (occurs in the brain) rather than peripheral process..


QUESTION 5
Please explain the difference in definition and treatment between Autoimmune Hepatitis and PBC? 
Since PBC is thought to be an autoimmune disease is AIH another distinct disease?  How can you have both?  Does treatment differ?

QUESTION 5
PBC and autoimmune hepatitis are different diseases.  They have in common that the two diseases are thought to be autoimmune in nature and may sometimes both occur in the same individual (overlap syndrome).  Though PBC is thought to be an autoimmune disease, medications used to suppress the immune system, that would be used in autoimmune hepatitis are generally not effective in PBC.  Some believe that these diseases may span a spectrum of the same disease.  My approach is that they are both autoimmune disease.  For PBC the target of the immune system are the bile ducts within the liver and in autoimmune hepatitis, the target is the liver cell itself..


QUESTION 6
I read very little about varices in the PBC digest. Is this common?
Does the bleeding from varices occur through the rectum or mouth?
If rectal how would a person distinguish from the varices or polyps?

QUESTION 6
Asymptomatic varices are quite common in PBC.  They may even occur in individuals with early disease.  Varices may occur in various parts of the body including the esophagus, stomach and rectum.  The most likely site that will rupture are the ones in the esophagus.  Blood may come out of the mouth and/or rectum.  

 

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