Primary Biliary Cholangitis (PBC) – What is it and Who is at Risk? [transcript] [audio]

Guest: Dr. Nancy S. Reau

Presenter: Neal Howard

Guest Bio: Dr. Nancy S. Reau is a hepatologist at the Rush University Medical Center in Chicago, specializing in Transplant Hepatology (primary specialty) and Gastroenterology. She is educating about Primary Biliary Cholangitis (PBC), which is a progressive autoimmune disease that affects the bile ducts in the liver. While PBC is a rare disease, affecting approximately one in 1000 women over 40, it has been the second leading overall cause of liver transplant in women in the U.S. since 1988. Dr. Reau is the Associate Director of Solid Organ Transplantation and Section Chief of Hepatology at Rush University Medical Center. She has over fifteen years of experience in hepatology, gastroenterology, and internal medicine, and specializes in treating a variety of complex liver disease, including Hepatitis B, Hepatitis C, liver cancer, liver transplant, and viral diseases. She has authored or co-authored over 100 peer-reviewed articles, invited reviews, editorials and on-line programs, as well as several book chapters, and two books.

Segment Overview: Dr. Nancy Reau, MD, Associate Director of Solid Organ Transplantation and Section Chief of Hepatology at Rush University Medical Center, discusses Primary Biliary Cholangitis (PBC), a progressive autoimmune disease that affects the bile ducts in the liver. She also talks about health care provider education, and the importance of routine liver testing.

Transcript

Neal Howard: Hello and welcome to Health Professional Radio. I’m your host Neal Howard. Glad that you could join us. Our guest is Dr. Nancy Reau. She’s Associate Director of Solid Organ Transplantation and Section Chief of Hepatology at Rush University Medical Center. Joining us on the program to talk about primary biliary cholangitis or PBC. It’s a progressive autoimmune disease that affects the bile ducts in the liver. Welcome to the program Dr. Reau.

Dr. Nancy Reau: Thank you so much for having me.

N: Now, I’ve given our listeners your title. Give us a little bit of your background, where you’re practicing and a little bit above and beyond your expertise as a hepatologist.

R: Absolutely. So most liver specialists go through internal medicine followed by GI training then do more training in liver diseases. I’ve finished all of my trainings in almost two decades ago. I have been working in an academic center since. I’ve been at Rush University Medical Center for about three years where I run the liver section and there is a Chicago big tertiary care program, so we see all sorts of liver diseases. We have a transplant program. So we are fortunate enough to have a wonderful opportunity for those people who have progressive liver disease where we can’t control or offer them a curative option at least in their life through organ transplantation.

N: Primary Biliary Cholangitis or PBC, what exactly is it and I know it affects the liver but how does it differ from some of the other liver conditions that you may see on a daily basis?

R: So PBC luckily is not one of the most common conditions we see. Also luckily, it does have a therapy that can stabilize and offer great prognosis to our patients. But unlike some of our diseases where we have a known trigger such as a virus or we know that it’s linked to something like obesity or diabetes, PBC is where your immune system suddenly targets something that doesn’t like it. For instance a war to that place that leads to destruction. In PBC, that war occurs at the small bile ducts. Your bile duct kind of look like a tree where the branches go from bigger and bigger, I mean big branches to smaller and smaller branches. And PBC starts by pruning or targeting the little tiny branches. Just like any tree in your backyard, if you cut enough of the big branches off, it can’t actually do its job. It’s going to kill the tree. And so for PBC, it becomes progressive when you lose the amount of bile necessary to do your liver’s function. So as they lose the bile duct, the liver doesn’t have the capacity to process and do all the jobs that it needs to with the bile and that leads to injury and scar tissue and overtime, cirrhosis.

N: Who’s at risk or PBC? Does it affect men and women equally?

R: No. PBC is highly more found in women and not men. Although I do have male patients. We probably assume it’s a combination of an environmental trigger and a genetic predisposition. So we know that most individuals with PBC might find someone else in their family or they themselves might have another immune disorder or they have a thyroid problem or a cause of lupus but you get the sense that there’s something that makes their immune system slightly more responsive. And then some unknown, although there are many hypotheses, some unknown trigger turns on that immune response and our goal is to identify that as early as possible so that we can keep that response from injury in the liver.

N: Now identifying it as early as possible, are there some symptoms that are glaring or is this something that is trial and error hit and miss as far as properly diagnosing PBC?

R: Unfortunately, most liver diseases, they don’t have very many symptoms until they have progressed significantly. When you look back the things we hear, the most are fatigue, may be mild belly pain, maybe some digestive issues. With PBC, we know some of our patients will also get itchy, especially on their hands and their soles before the disease is recognized. But often are found through routine labs. You go to your family doctor on an annual basis. Blood tests show some liver test abnormalities that leads to another additional information or hopefully leads to a search for why those numbers might be not at the normal range and that eventually comes to a conclusion of PBC.

N: Are there any I guess misconceptions surrounding PBC because it is a liver disease and since it falls in that category, do some people think that they’ve got hepatitis C or B when they’ve actually got PBC?

R: I think that anything that links to the liver, the number one comment my patients will tell me when they’re first diagnosed is, “I can’t have liver disease. I don’t drink.”. Liver injuries can occur from multiple different insults and PBC is not the only immune problem that can occur. However once you’re told that you have a liver disease, most of my patients are confused by things that are more common such as alcohol injury or hepatitis C because there’s a lot of recognition about our first cohort in respect to hepatitis C. There have been a couple of outbreaks of hepatitis A. And so anything that they perceive up is a liver injury (crosstalk).

N: Now once you suspect that someone has this condition, what types of tests are we talking just blood tests or their other types of tests to determine the condition and the severity of it?

R: Right. So luckily PBC has an immune marker that occurs in the vast majority of the affects in individuals and if we have an immune marker which is known as anti-mitochondrial antibody, we’re pretty sure that we have PBC. You don’t necessarily do a lot of invasive tests, but every patient needs to be staged. So we want to know how affected your liver disease is. We can often do that through blood tests and imaging or more sophisticated studies that look at stiffness in the liver. The other thing that we also concentrate on other risk factors outside the liver, for PBC patients are at increased risk for bone disease, osteoporosis and osteopenia. They might develop thyroid problems over their lifetime. There’s a suggested association with celiac disease. They might have sickle syndrome which is poor amounts of saliva or dry eyes. There’s an increased risk for gum disease, dental decay, corneal abrasion. So once you label someone or you identify them with PBC, it comes with a bucket of other screening and monitoring, not just liver.

N: Once you’ve been diagnosed properly, is this a lifetime management thing? Can it be reversed or is a transplant imminent?

R: Well I think, the first point is it is a lifetime condition. Since it’s your immune system until we can figure out how to reset your immune response, we know that we’re going to have to do something for the rest of your life. That being said, there are therapies that are very effective for PBC and if you find something early and you stabilize it, the medications can actually allow you to have a long, essentially the same quality and length of life as someone who doesn’t have PBC, but the point is to find it early. Some are not lucky enough to respond and so if that’s case, then sometimes they do progress and require transplant or we find them late and transplantation offers them the best long term prognosis. But the hope is really to start intervention well before that. And then we luckily have 5 chemical markers. They can watch your lab to see if the medications are working for you.

N: Now where can we go and learn more online about this condition?

R: There are lots of great resources for PBC. There are advocacy groups such as the PBCers, our company that produce the medications that can intercept and have great resources, and then places like the American Liver Foundation which is another advocacy, have great resources. In addition, things like the CDC and NIH also have great pieces of appropriate resources and a lot of our big academic institutions will have information for patients that you can Google. The most important thing to recognize is that when you do look something up, you can get a lot of information that may not be relevant to you. So kick those questions, don’t be scared by them, but you can see your physicians. Ask those things that sound like something you didn’t discuss in the office. Don’t just give up hope or get frustrated.

N: Well Dr. Reau, it’s been a pleasure. Lots of great information about primary biliary cholangitis. I’m hoping that you’ll come back and talk with us and give us some more information.

R: Wonderful. Anytime. Thank you so much for having me.

N: You’ve been listening in the Health Professional Radio. I’m your host Neal Howard in studio with Dr. Nancy Reau, Associate Director of Solid Organ Transplantation and Section Chief of Hepatology at Rush University. Transcripts and audio of the program are available at healthprofessionalradio.com.au and also at hpr.fm.

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