DOCTORS PANEL QUESTIONS & ANSWERS

 

 

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: 
Howard J. Worman, M. D.
Associate Professor of Medicine and Anatomy and Cell Biology
College of Physicians and Surgeons
Columbia University
New York, NY 10032

2004


QUESTION 1
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?

ANSWER 1
"Varices" (I assuem you man varicose veins between the systemic and portal circulations) occur in farily advanced cirrhosis.  They can occur in subjects with PBC when  cirrhosis (scar formation and nodules in the liver) has develops.  If the pressure in the portal circulation (the circulation of the gut that then goes to the liver) increases because of cirrhosis, varicose veins can form in the stomach and esophagus (gastric and esophageal varices) and rectum (hemorrhoids). Gastric and esophageal varices can rupture and bleed massively. The subject will generally vomit blood or "coffee grounds," which is partially digested blood.  Esophageal varcies can be diagnosed by upper endoscopy (insertion of a fiber optic tube into  the esophagus and stomach) and rectal varices by flexible sigmoidoscoy or colonoscopy (insertion of a fiber optic tube in the rectum).  Sometimes rectal varices can be felt on manual examination.  Rectal varices are very different from polyps; if they bleed, they generally bleed more than polyps bleed.


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

ANSWER 2
Since "autoimmune cholangiopathy" is a very ill-defined condition (if it really is a condition), I cannot answer this question.


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

ANSWER 3
You should work closely with a good doctor who is familiar with the condition.  You should keep a very positive attitude as, in many or most cases, having PBC should not significantly effect your daily life.  You should realize that, if it becomes necessary, liver transplanation is available as a "life insurance policy" for most individuals with PBC.


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?

ANSWER 4
Nobody really can answer this question.  Fatigue may occur in any subject with chronic liver disease, not only PBC.  Subjects with other chronic diseases also often suffer from fatigue.  Nobody knows why.  It is not clear why some subjects with PBC are sometimes more fatigued than others.  In subjects with chronic liver disease, it is not clear what is "normal" fatigue (i.e. fatiuge anyone may suffer from working too hard and being too busy) and what may result from the disease.


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?

ANSWER 5
Autoimmune hepatitis (AIH) and PBC are distinct diseases.  In the vast majority of cases, PBC and autoimmune hepatitis can be readily distinguished by an experienced doctor.  PBC affects the bile ducts within the liver and AIH primarily affects the hepatocytes, or major cell type in the liver.  While some doctors sometimres refer to "overlap" syndromes between PBC and AIH, this is an ill-defined condition and, if there is "overlap," it occurs infrequently.  Treatments are different.  For AIH, treatment is generally immunsupression (prednisone or a similar drug with or without azathioprine).  For PBC, the treatment is ursodiol. 


QUESTION 6
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 6
First of all, "LFTs" are a terrible thing to call serum ALT, AST and alkaline phosphatase activities.  They do not really tell you about "liver function."  Regarding your question, there are many causes of "cholestatic LFTs" (I assume you mean elevated serum alkaline phosphatase activity).  In most cases, elevated serum alkaline phosphatase actvity does not indicate PBC but another problem.  The problem may not always be diagnosed by liver biopsy.  If someone's mother has PBC, it is still very unlikely that she will have PBC (however, probably a bit more likely that for an unrelated person).

 

 

 

 

BACK