MISCELLANEOUS QUESTIONS


Andrew Mason, MBBS MRCPI
Associate Professor of Medicine
Division of Gastroenterology, Department of Medicine,
University of Alberta, Edmonton, Canada
December 2002

Question
My doctor, who is not hepatologist admits he knows very little about PBC.

Answer
Well most of us Hepatologists don't understand the disease very well.

Andrew Mason, MBBS MRCPI
Associate Professor of Medicine
Division of Gastroenterology
Department of Medicine, University of Alberta
Edmonton, Canada T6G 2
December 2002

Question
After symptoms begin to surface in the individual, how many years of good health might one expect to have, or is this totally based on the individual?

Answer
It used to be thought that PBC was a progressive disease that would lead to liver transplant in 9 to 15 years. Now we know that many patients with mild disease have no progression to cirrhosis or a need for liver transplant.

Dr. Howard Worman
Division of Digestive and Liver Diseases
Departments of Medicine and of Anatomy and Cell Biology
College of Physicians & Surgeons
Columbia University
New York, NY 10032
December 1999

Question
Hepatic encephalopathy....once this happens will we have to take the medicine everyday until we get the transplant? Information says the toxins damage the brain, when will the brain go back to normal or does it ever?

Answer
Hepatic encephalopathy occurs in individuals with failing livers. In individuals with PBC, it usually occurs once the patient has advanced cirrhosis. Once liver damage in cirrhosis is bad enough to cause hepatic encephalopathy, it is generally not reversible. For this reason, most patients will have to take medications to prevent encephalopathy until they receive a transplant. An exception may be those with reversible situations that exacerbate encephalopathy, such as gastrointestinal bleeding or infection.

Dr. Howard Worman
Division of Digestive and Liver Diseases
Departments of Medicine and of Anatomy and Cell Biology
College of Physicians & Surgeons
Columbia University
New York, NY 10032
April 2000

Question
Do you know of any studies being done on benign tumors in the liver? Can these type of tumors become malignant? What effect, if any could these tumors have on a person with PBC or other autoimmune liver disease?

Answer
There are several different benign tumors of the liver. The most common is probably cavernous hemangioma. Others include ademonas and hamartomas. Benign tumors do not become malignant. The occurrence of a benign liver tumor in an individual with PBC or another autoimmune liver disease is probably coincidental. I am not aware of any studies showing that benign liver tumors are associated with PBC or other autoimmune liver diseases. I also do not know of any data showing that these tumors have any influence on disease progression or outcome in PBC.

Melissa Palmer, M.D.

Specialty: Gastroenterology and Hepatology

Private Practice Long Island, NY

01/25/04

QUESTION

Is it dangerous for a person with PBC to get pregnant?  Is there anything specific to watch for or do?

ANSWER

Women with primary biliary cirrhosis generally have uneventful pregnancies and deliveries.

However, some studies have noted a greater-than-average incidence of stillbirths, spontaneous abortions (miscarriage), and worsening liver function among these women. These events more commonly occurred in women with advanced liver disease - stage 4 disease.  Cirrhosis (stage 4 PBC) is often associated with amenorrhoea (lack of menses) and infertility. Consequently, women with stage 4 disease-especially decompensated cirrhosis-may have difficulty conceiving. As a result of their advanced liver disease, women with decompensated cirrhosis who do conceive have an increased risk of serious complications during pregnancy.

Bleeding from esophageal varices is probably the biggest pregnancy-related health risk for women with decompensated cirrhosis. Variceal bleeding is most common during the second trimester, occurring in approximately 20 to 45 percent of women with portal hypertension. Ten percent of the time, women with decompensated cirrhosis experience variceal bleeding during labor and immediately after childbirth. Death of the mother from uncontrollable variceal hemorrhage occurs approximately 10 to 18 percent of the time during the course of pregnancy. Depending on the trimester, the baby may nevertheless have  chance for survival. Women with decompensated cirrhosis who are thinking about becoming pregnant should undergo an upper endoscopy, which can assess the presence and degree of esophageal varices.

Pruritus can worsen during pregnancy and may be successfully and safely treated with cholestyramine. On the other hand, pruritus sometimes improves during pregnancy. Ursodiol is generally considered to be safe during pregnancy.

END