Ask the PBC doctor

DISCLAIMER: The following is intended for general information purposes only. It is not intended to be comprehensive. It does not in any way constitute legal or other professional advice, and should not be relied upon as such. The reader is cautioned to consult their own physician and other experts for advice regarding specific health concerns. The Canadian PBC Society is not responsible for the accuracy, completeness, or any action taken on the basis of the information mentioned herein. It is not intended to substitute for and/or supersede one’s own physician’s advice.

Symptoms and Symptom Management

Is fatigue a common symptom of PBC and is there any medication to treat it?

Fatigue occurs in up to 80% of patients with PBC. However, on diagnosis up to half of the patients that we see in clinic have no symptoms because they are generally picked up on routine screening with abnormal liver tests. Patients with fatigue often have to sleep during the day or find they have little energy to do much in the evenings. This symptom is usually persistent, although a small proportion of patients do respond to the newer medications being studied. While obeticholic acid does not appear to improve either malaise or brain fog, some of the newly developed drugs known as PPAR inhibitors appear to improve itching and sleep in a small proportion of patients with PBC. Whether these medications truly improve fatigue, itch and sleep will be better understood once the medications become available for treatment of PBC.
— Dr. Andrew Mason, 2023

Is nausea a common symptom of PBC and can anything be done about it?

Nausea is not a common symptom of PBC but stomach issues may occur in small proportion of patients. Nausea is usually associated with intestinal problems and a common side effect of taking medications. Fortunately, both ursodiol and cholestyramine seldom cause nausea and both drugs are generally well-tolerated. Other medications may cause nausea, especially on an empty stomach. For example, multivitamins can cause a degree of nausea if not taken with food. Common intestinal diseases associated with nausea include peptic ulcer disease, irritable bowel disease as well as other inflammatory bowel conditions. If nausea is a significant problem, it should be discussed further with your doctor.
—Dr. Andrew Mason, 2023

Do problems with clotting start in the early stages of PBC?

It is unusual for clotting problems to be present in the early stages of PBC unless it is linked with another disease where clotting is a problem. Clotting problems generally start when patients have cirrhosis, which is associated with low platelets as well as a diminished production of clotting factors from the liver. However, sometimes patients with PBC can have problems with absorbing their fat-soluble vitamins – A, D, E and K. Vitamin K deficiency is associated with lack of production of clotting factors. So if patients have a lack of vitamin K early on in their disease, they could theoretically develop clotting problems. I recommend that patients at risk of nutritional deficiency have levels of specific vitamins assessed by their doctor. I usually recommend all PBC patients take an age/sex appropriate multivitamin as well as 2,000 IU vitamin D depending on their levels to avoid these problems.
—Dr. Andrew Mason, 2023

Is hepatic encephalopathy of PBC corrected or improved following successful liver transplant?

Hepatic encephalopathy (HE) is a deterioration in brain function observed in people with acute liver failure of chronic liver disease. Following a successful liver transplant, this is pretty much improved in all patients, but some issues related to encephalopathy may remain if severe prior to transplantation. Another consideration is that the liver transplantation does not necessarily reverse the chronic fatigue experience by patients with PBC. There are other things to consider as patients may have problems with concentrating or thinking due to conditions unrelated to PBC. If this is the case, a degree of neurological disease may continue following transplantation. Also, following liver transplantation some of the drugs used for immunosuppression can make patients concentration a little fuzzy.
—Dr. Andrew Mason, 2023

Why do some patients experience aching joints?

PBC and other autoimmune diseases in general, share several features in common. For example, patients with PBC may experience symptoms that patients with other rheumatological diseases experience as well. Patients with PBC and Sjogren’s syndrome experience dry eyes and dry mouth. Patients with PBC also experience aching joints and aching muscles that patients with rheumatological diseases experience. In fact, this is not uncommon in patients with other forms of liver disease such as hepatitis C virus infection, hepatitis B virus infection and hemochromatosis (an iron storage disease) who also experience aching joints. If the pain is not too severe and the joints are not swollen or deformed, we usually recommend taking Tylenol in regular doses to treat aching joints. If the joint problems are severe, we often look for other causes, such as rheumatological disease.
—Dr. Andrew Mason, 2023

Is unsteadiness on the feet a common occurrence with PBC and, if so, why does it occur?

It appears that some patients with PBC have swings in their blood pressure due to changes in the autonomic nervous system that regulates involuntary physiological processes. Sometimes their blood pressure is high and sometimes their blood pressure is low. This means that when you stand up too quickly, the blood supply to the head can be reduced and patients can become unsteady on their feet and dizzy. It is not known what causes this change in the nervous system controlling the blood pressure; and this is subject to research. Unsteadiness on the feet may occur for several other reasons, and if this is becoming a problem, then patients should see their doctor to be worked up for other causes of unsteadiness that include neurological or potentially cardiac causes or disease.
—Dr. Andrew Mason, 2023

Is itching a common symptom of PBC and how is it described by those experiencing it?

Itching in PBC is truly an individual experience. In studies including large numbers of PBC patients itching has been reported to occur in roughly 13-48% (K Ismond et al Can Liver J; 2019; S. Rice et al. Clin Gastro Hep. 2021), but in most patients it has minimal impact on their quality of life (GF Mells et al. Hepatology 2013). For those that experience it, it’s described as a burning, tingling sensation in the skin. The itching associated with PBC is often felt on the soles of the feet and palms of the hands but may be experienced on any part of the body. Everyone’s experience differs, but it can be profound and life altering in some patients. If you are itching to the point of scratching at night to cause bruising or skin bleeding you should contact your treating physician to explore anti-itch therapies which can often really help. First line treatments include skin moisteners and cholestyramine powder. For more significant itch, medications such as rifampin, naltrexone, sertraline and bezafibrate can be effective.
—Dr. Mark G. Swain, 2023

Is there a relation of rectal bleeding to PBC?

In general, no, unless there are hemorrhoids which may rarely be related to liver disease. In advanced PBC in people with cirrhosis and portal hypertension (increase in pressure in the veins that drain from the gut through the liver to the heart), rarely rectal varices can develop. If this happens, and they bleed, the bleeding can be quite dramatic and warrants a visit to the nearest hospital emergency.
—Dr. Mark G. Swain, 2023

Is there a link between pruritus and restless leg syndrome and PBC? Is there a diet or supplement to alleviate this?

Pruritus is an intense itching sensation that produces the urge to rub or scratch the skin to obtain relief which may be a symptom of PBC. Although restless leg syndrome is not directly related to pruritus, it may make it worse. For restless leg syndrome a number of anecdotal reports have suggested that supplementation with vitamin D (most PBC patients should typically be taking vitamin D 2000 IU/day for bone health anyway), vitamin E, calcium and magnesium may be beneficial.
—Dr. Mark G. Swain, 2023

Are telangiectasias related to PBC?

Telangiectasias are small blood vessels that sit near the skin’s surface and widen, creating visible patterns of lines. Often referred to as spider veins or spider telangiectasia, they can occur anywhere on the body or face. They can be related to PBC, typically in the setting of more advanced liver scarring/cirrhosis, but are also common in people without liver disease.
—Dr. Mark G. Swain, 2023

How do you pace your daily activities while working full-time to preserve stamina and energy so you won’t feel exhausted at the end of day? Will working full-time or being fatigued all the time make your PBC progress faster?

These are very good questions and important issues for PBC patients experiencing fatigue – the most common symptom in PBC patients. Given that the cause of fatigue in PBC is unknown, recommendations to cope include resting when the situation allows, getting a good night’s sleep, and keeping as aerobically fit as possible. Ensuring a well-balanced diet and maintaining a healthy weight will also help with energy levels. Make sure your doctor has ruled out other common causes of fatigue which are specifically treatable (e.g. anemia, depression, diabetes, adrenal impairment, thyroid disease). Sometimes, a full-time job is no longer possible, and a modification of your work schedule may be needed (i.e. part-time, working some from home). Coffee can be consumed to improve energy and concentration and is broadly considered to be healthy for the liver. There is no clear link between the severity of liver disease in PBC and the severity of fatigue (or tiredness) that people experience. There is no evidence that working while being fatigued impacts on the progression of the liver disease.
—Dr. Mark G. Swain, 2023

What suggestions do you have for women with PBC going through severe menopausal symptoms (e.g. hot flashes, sleeplessness, mood swings)?

Hormone replacement therapy (HRT) can be used in women with liver disease, but caution might be needed if there is a history of severe itch from your PBC, or your PBC is very advanced. Therefore, it is generally okay to discuss your symptoms with your primary care and consider HRT if it best helps your needs.
— Dr. Inbal Houri, 2023

Should someone with PBC swim in a chlorinated pool?

Yes, swimming is an excellent form of exercise that offers a full-body workout with numerous benefits to both physical and mental health. As a non-weight bearing exercise, it provides cardio workout without putting pressure on the joints and bones. However, for some PBC patients suffering from dry eyes, mouth and skin the chlorine may increase symptoms, in which case using moisturizer, swim goggles and preservative-free eye drops may help. Prior to starting any exercise program, it is important to consult with your physician.
— Dr. Inbal Houri, 2023

Related Disorders and Causes

Are there any irritable bowel type symptoms associated with PBC?

There are no irritable bowel type symptoms associated with PBC. Irritable bowel symptoms such as bloating, abdominal discomfort, mild diarrhea or constipation are common in healthy people. PBC can occasionally be associated with mild upper-right abdominal discomfort. Additionally, some PBC patients may have celiac disease (an autoimmune disease). If you have symptoms such as diarrhea, weight loss or significant abdominal pain – please consult your physician.
— Dr. Inbal Houri, 2023

Is it true that one does not get PBC without some other so-called autoimmune disease?

No, having PBC does not necessarily mean you will get another autoimmune disease. However, there is a higher risk for additional autoimmune diseases in PBC patients and their family members. Those typically associated are thyroid disease, Sicca syndrome/Sjogrens, Raynaud’s phenomenon, rheumatoid arthritis, and celiac disease.
— Dr. Inbal Houri, 2023

Is the risk for osteoporosis higher in patients with PBC?

Yes, PBC patients have a higher risk of developing osteoporosis. Consult your physician about the appropriate screening schedule and recommendations for vitamin D or calcium supplements.
— Dr. Inbal Houri, 2023

Is there a higher incidence of kidney stones among PBC patients?

I am not aware of any published reports on an increased frequency of kidney stones in PBC patients. However, theoretically, PBC patients do have a higher incidence of something called distal renal tubular acidosis (a kidney acid-base defect) and this has been associated with an increase in renal stone formation.
—Dr. Mark G. Swain, 2023

Has there been any indication that nail polish may either increase your chances of developing PBC or cause PBC?

This is a very interesting and controversial question. In a patient interview study from Dr. Eric Gershwin’s group in California (published in Hepatology, 2005), exploring potential lifestyle risks for PBC, they identified frequent use of nail polish as slightly increasing the risk for the development of PBC. Importantly, this increase was very slight.
—Dr. Mark G. Swain, 2023

Has there been any documented cases of reversal of liver damage?

The reversal of liver scarring in PBC by therapy is controversial. Dr. Marshall Kaplan in Boston has described patients in the literature who appear to have had remarkable reversals of liver scarring when treated with ursodiol (Urso) plus methotrexate. However, these findings are not broadly accepted, and in general the liver scarring which occurs in PBC should be viewed as irreversible. The goal of current therapy is to stop the liver scarring associated with PBC from progressing.
—Dr. Mark G. Swain, 2023

How does PBC affect the body in combination with other autoimmune diseases such as Graves’, diabetes, arthritis and ulcerative colitis? Which specialist could best address the problem of these conditions and their associated medications that might impact PBC?

Autoimmune diseases commonly occur together in patients and in families probably as a result of genetic susceptibility to autoimmune disease in general. Each autoimmune disorder described affects the body in different ways. The main problem with having PBC and liver disease is that it affects the absorption of fat-soluble vitamins, and therefore patients with PBC are more likely to develop thinning of the bones as they do not absorb the calcium and vitamin D. PBC may also affect energy levels and cause itching and dry eyes with dry mouth. The other diseases such as Graves’ and diabetes may have some effect on heart function, and patients with diabetes can get blood vessel disease as well as kidney disease if their control of sugar is not good. Patients with ulcerative colitis may get disease of the skin with rashes, as well as arthritis. Generally, if you have a specific disease that is difficult to manage, it is always worth seeing a specialist associated with that disease such as a rheumatologist for arthritis, and endocrinologist for diabetes and Graves’ disease, a liver specialist for PBC, or a gastroenterologist for ulcerative colitis. However, if the diseases are well controlled, it may be simpler and more coordinated to see one physician who is familiar but not an expert in all these diseases.
— Dr. Andrew Mason, 2023

Medications, Supplements and Treatment

What supplements are recommended for PBC patients?

We usually recommend that patients take an age-appropriate multivitamin, as well as supplements to prevent thinning of bones and osteoporosis. This includes vitamin D 2,000-4,000 IU/day depending on blood levels, and an elemental calcium 500 mg supplement. Discuss taking calcium with your pharmacy as different formulations provide different amounts of elemental calcium. Excessive calcium supplementation should be avoided due to the risk of buildup of calcium in blood vessels and the development of kidney stones.
— Dr. Andrew Mason, 2023

Is there a safe cholesterol medication to take?

Most cholesterol medications are safe for people with PBC. The statins, such as Lipitor, rarely cause any changes in liver function tests. As patients with PBC have their liver function tests done regularly anyway, we can usually spot when patients are having a reaction to this treatment. Also, cholesterol medications such as fenofibrates may also be of benefit to reduce cholesterol and triglycerides in patients with PBC. More recently, studies using bezafibrate have actually shown benefit in treating PBC by reducing liver tests and improving itch. The only drug that reduces cholesterol that may be bad for PBC patients is niacin, which causes a dose-dependant damage to the liver. Patients who have high cholesterol and other risk factors for heart disease such as smoking, hypertension, diabetes, or a family history of heart attacks at a young age could be considered patients for cholesterol treatment.
—Dr. Andrew Mason, 2023

What is the risk for us getting or not getting hepatitis shots?

Infants in Canada get vaccinated for hepatitis B virus infection. Patients who travel to tropical countries may be exposed to hepatitis A. Patients that have sexual relations or exposure to blood products from patients with hepatitis B may be at risk of getting it. Those with liver disease may have worse reactions if they get hepatitis A or B so we recommend patients get vaccinated for both if travelling.
—Dr. Andrew Mason, 2023

At what T score level should a PBC patient with a low bone density consider a bisphosphonate a necessity?

There is not an absolute T score. This score represents a variance (standard deviation) of bone mineral density and osteoporosis with severe thinning defined as -1 to -2.5 reduction. In general, fracture risk increases two to three times with each -1 reduction in T score. Therefore, all PBC patients should have their serum 25-OH-vitamin D levels checked as well as bone mineral density. Although a proportion of patients have normal bone mineral density, I recommend all PBC patients should be on calcium and vitamin D 2,000-4,000 IU/day to prevent bone loss. If people have a low bone density despite taking vitamin D and calcium, then a bisphosphonate treatment can be considered. We could either use a drug such as Didronel to inhibit the breakdown of bone, the stronger bisphosphonates such as Fosamax or Pamidronate infusions. Hormone replacement therapy is no longer recommended for patients with progressive liver disease.
—Dr. Andrew Mason, 2023

Do cholesterol drugs affect liver function tests?

Cholesterol drugs are often required in patients with PBC. This is because when bile ducts are blocked, the cholesterol cannot get out of the liver and it tends to build up in the body. We know that cholesterol can lead to problems with coronary artery disease and therefore we do recommend anti-cholesterol therapy for patients that are at risk for coronary artery disease, heart attacks and strokes. The drugs that are referred to as “statins”, such as Lipitor, are commonly used in the population to combat cholesterol. These medications are very safe. On occasion, they can cause an increase in liver function tests. However, as patients with PBC are monitored regularly for their liver function tests, it is usually quite safe for them to take these medications. If their liver tests become worse on the statin, then the dosage can be modified or the drug can be discontinued. There is also data suggesting that other lipid (fat) lowering agents such as fenofibrates positively impact on the progression of PBC. These medications being tested in clinical trials are relatively safe and can potentially improve PBC. The difference between fenofibrates and statins is that the fenofibrates can also reduce other fats such as triglycerides as well as cholesterol in the body.
—Dr. Andrew Mason, 2023

Should PBC patients take vitamin B3? Is niacin and niacinamide the same thing; or if different, is one safer?

Niacin is a water soluble vitamin also known as vitamin B3. There are different formulations including nicotinic acid and nicotinamide. Nicotinamide is generally better tolerated than nicotinic acid and does not generally cause blushing, however nausea, vomiting and signs of liver toxicity may occur when high doses of the supplement are taken. This may be observed with doses of 3 g/day. The current recommendation is to take about 20 mg/day. Patients usually receive enough niacin in meats, poultry, fish, cereals, vegetables and seeds. Milk also provides niacin as well. Previously, niacin was used to treat high cholesterol, but due to liver toxicity, newer medications are preferred such as “Statins”. Personally, I do not recommend additional vitamin B3 for patients more than can be found in standard multivitamin preparations. Accordingly, I advise most patients with PBC to take a multivitamin with extra vitamin D 2,000–4,000 IU/day as well as calcium to avoid bone disease.
—Dr. Andrew Mason, 2023

Do you recommend vitamin B12 shots for PBC fatigue; and if so, are they safe?

Vitamin B12 shots are safe and no toxicity or adverse affects have been associated with large doses. Vitamin B12 deficiency is estimated to affect 10 to 15% of individuals over the age of 60 and is specifically associated with pernicious anemia, which is also an autoimmune condition. Deficiency is also seen with vitamin B12 malabsorption. Both these conditions are distinct from PBC; and therefore, I do not generally recommend vitamin B12 shots for fatigue in PBC patients. However, up to 5% of patients with PBC can develop the malabsorption syndrome celiac disease that can be associated with low vitamin B12 levels. I would consider evaluating the levels of vitamin B12 in patients with (i) low hemoglobin, (ii) antibodies to intrinsic factor which helps with vitamin B12 absorption, (iii) signs of pernicious anemia and vitamin B12 deficiency with sore tongue, tingling in arms and legs and other neurological symptoms. If the vitamin B12 level is low – I would recommend the use of B12 shots. As part of your work-up for PBC, your doctor may arrange a gastroscopy, look for signs of inflammation in the stomach and may also check other autoantibodies associated with autoimmune conditions that are linked with vitamin B12 loss.
—Dr. Andrew Mason, 2023

Is it safe for a PBC patient to be pregnant? Should she continue to take Urso?

In general, yes. If a PBC patient has cirrhosis it may be difficult for them to get pregnant; and if they do, there may be increased problems. Urso is felt to be safe in pregnancy and should be continued while pregnant unless directed otherwise by your liver doctor. I recommend that my patients currently taking Urso not to stop during pregnancy. Urso can be taken while breast feeding and will not harm the baby.
—Dr. Mark G. Swain, 2023

Are asthma medications harmful to the liver?

Asthma medication choices have expanded dramatically in the past decade. The asthma medications montelukast and zafirlukast have been reported to rarely cause significant liver injury and it is recommended to follow liver blood tests regularly if you are taking these medications. However, in general asthma medications are not harmful to the liver
—Dr. Mark G. Swain, 2023

Is there any difference between the generic and non-generic forms of Urso (UDCA)? What about the non-active ingredients?

In general, most people with PBC can use generic and non-generic forms of ursodeoxycholic acid (UDCA) interchangeably. However, the non-active ingredients are different, and this may theoretically lead to altered pharmacokinetics (i.e. rate of absorption and plasma levels) of the two Urso compounds. In addition, I have noted that some patients may experience intolerance to some of the non-active ingredients (e.g. rash, diarrhea) in the generic forms. In general; given the cost differential, I typically support using the generic Urso first, and if it is effective biochemically (i.e. the liver tests fall in newly started patients, or do not rise after switching patients currently taking non-generic Urso to an equivalent dose of generic Urso) and does not cause any adverse side effects or symptoms, I continue on with the generic form.
—Dr. Mark G. Swain, 2023

Could the drugs Lyrica or Cymbalta be used for pain in patients with PBC?

Lyrica was originally developed as an anti-seizure medication and Cymbalta an anti-depressant. However, both these drugs have been approved in the USA to treat neuropathic pain (i.e. pain coming from diseases which affect or damage nerves; such as diabetes). In addition, Lyrica can be used for the treatment of pain associated with fibromyalgia. In general, these are “relatively safe” drugs and could be used in the setting of PBC (after discussion with your family doctor) if you are suffering from one of these conditions associated with pain and you have no other contraindications to their use.
—Dr. Mark G. Swain, 2023

Does being on Urso medication to treat one’s PBC put you at risk for getting kidney stones?

I am not aware of any association between Urso and kidney stones. I would not recommend stopping Urso because of kidney stones.
—Dr. Mark G. Swain, 2023

Can Urso lead to stomach problems such as irritation, pain, cramps, etc.?

Yes. Urso is a bile acid and as such can cause stomach irritation. Taking it with food, in divided doses, or at night before bedtime can often improve this.
—Dr. Mark G. Swain, 2023

Which gastroesophageal reflux disease (GERD) medication is least likely to cause a negative interaction with Urso?

The different medications available for GERD do not interact with ursodiol and are not contraindicated in patients with PBC. Please consult your family doctor regarding the most appropriate choice for you.
— Dr. Inbal Houri, 2023

Have you heard or seen adverse reactions to Urso characterized by widespread thickened skin eruption?

No. Very rarely, people can have an allergic reaction to Urso, but this usually causes hives, rather than a rash with thickened skin. In most cases of an allergic reaction to Urso other treatment options are used instead. In rare cases, people who have an allergic reaction to Urso may be able to undergo a procedure called “desensitization” which can only be done under the guidance of a trained allergist. Other skin changes should be investigated.
— Dr. Inbal Houri, 2023

Is it safe to take green coffee bean extract if you have PBC?

There is a growing market of herbal therapies and supplements. It is generally not recommended to take these as none have been shown to have proven benefit in PBC, and some can be harmful to the liver, even in healthy people. If you do wish to take an herbal therapy or supplement, please discuss this with your doctor, and also make sure you record the dates you take the supplement and how much you are taking. This way, if the supplements do make your liver tests worse, your doctor can keep track of what you were taking at the time.
— Dr. Inbal Houri, 2023

If a patient with high cholesterol, given a statin (Lipitor), develops high alanine transaminase (ALT) and aspartate aminotransferase (AST) levels in their bloodwork, is this considered a class drug reaction, or could they possibly safely change to another statin such as Crestor?

Statins are drugs used for high cholesterol and can cause high liver enzymes in all people, with PBC as well as those without PBC. The increase in liver enzymes is related to the medication. In most cases (depending on the background liver disease and enzyme levels) it is safe to try another statin according to medical advice, if followed closely by a doctor to monitor the liver tests on the new drug.
— Dr. Inbal Houri, 2023

For someone with a family history of stroke and/or heart attacks is there a concern with taking 81 mg/day of aspirin?

In general, there is no specific contraindication to aspirin in patients with PBC, and the majority of patients can use safely if needed. As with all medications, it is important to weigh the risks and benefits before initiating treatment. If your specialist or your family doctor has told you that you need aspirin, it is ideal if they communicate with your liver specialist if you have cirrhosis or advanced liver disease. People taking aspirin who have esophageal varices (enlarged blood vessels in the esophagus which can only be seen on endoscopy) or ascites (fluid in the abdomen due to advanced PBC), may occasionally be at higher risk for adverse effects, in which case an individualized assessment of risk-benefit should be made.
— Dr. Inbal Houri, 2023

Is there any slow release or long-acting form of Urso being considered for the future?

Ursodiol (Urso) can be taken once-daily or in a split dose (according to personal preference). Once-daily dosing is considered the standard for long-acting medications, and there are currently no different formulations available.
— Dr. Inbal Houri, 2023

With a greater risk of osteoporosis in patients with PBC, what amount of supplemental calcium (and in what form) do you recommend; if at all, for patients who are 50+ years old?

It is now known that it is better to take dietary calcium rather than calcium supplements, as calcium supplements may increase the risk of heart attack and stroke over the long run. The recommended daily calcium intake is 1000-1200 mg. We therefore usually work with patients to optimize dietary calcium intake before using supplements. For additional information, refer to: Calcium | Osteoporosis Canada

Vitamin D 1000-2000 IU/day is generally recommended. For some, higher doses are needed according to your physician’s recommendation.
— Dr. Inbal Houri, 2023

Are there medications to treat high blood pressure that are safe for those with PBC?

In general, all medications for high blood pressure are safe in people with PBC. In those with cirrhosis, personalized decisions should be made depending on liver disease complications and other regular medications with potential interactions.
— Dr. Inbal Houri, 2023

Is it safe to take Flexeril with Urso?

Flexeril (cyclobenzaprine) is a muscle relaxant for which there is no known interaction between it and Urso. This medication is not recommended in advanced liver disease, patients with cirrhosis should use only per doctor recommendations.
— Dr. Inbal Houri, 2023

Should PBC patients get a flu shot? Is it true that you could get the flu after receiving the vaccination?

The flu shot is recommended for anyone with cirrhosis, as well as other high-risk groups as defined per medical authorities. PBC diagnosis in itself is not a high-risk factor. There are many types of influenza (or flu) viruses. Vaccines are created to fight the most common flu viruses every year, but you may get a flu virus that the vaccine does not work for. If this is the case, you will get the flu. This does not mean that the vaccine did not work, it just means you got a type of flu virus that the vaccine does not fight. The flu vaccine does not make the flu worse, and it does not give you the flu.
— Dr. Inbal Houri, 2023

Is there a need to phase off cholestyramine or can you just stop taking it?

Cholestyramine is a medicine used to treat itch that is caused by diseases of the bile ducts. To stop taking cholestyramine, there is no need to phase or wean off this medication.
— Dr. Inbal Houri, 2023

Diagnosis, Testing and Research

Should children of PBC patients have their AMA checked?

We know that family members of patients with PBC have a 10 to 15-fold increased risk of developing the disease. However, we must consider that PBC is a rare disease. In North America, PBC occurs 1 in 10,000 to 1 in 50,000 people. This means an increased risk of tenfold translates to family members having a 1:1000 to 1:5000 risk of PBC. I usually tell patients that children do have increased risk and if they have increased liver function tests when they have their general physicals done, they should also have their antimitochondrial antibody (AMA) checked if their liver tests are abnormal. We also recommend having AMA checked if relatives of patients start to experience any signs and symptoms of PBC such as fatigue, pain in the right side of the abdomen, dry eyes, dry mouth or itching. Otherwise, we do not recommend the routine checking of AMA because a positive AMA with normal liver tests may occur and most of these subjects do not develop PBC.
—Dr. Andrew Mason, 2023

How does the AMA affect the liver? And other body structures?

Antimitochondrial antibody (AMA) is a diagnostic marker for PBC. Some patients with PBC, however, have no AMA in their blood. This only applies to a small percentage, approximately 10-20% of patients with PBC. It is unknown whether AMA does affect the liver or whether this is just a marker for disease. As patients with no AMA may get PBC, I suspect that AMA is not the cause of the liver disease, but a marker of PBC.
—Dr. Andrew Mason, 2023

For those already diagnosed with PBC, is a liver biopsy necessary; and if so, what is the purpose?

Most cases of PBC can be diagnosed without a liver biopsy. Diagnosis is made based on liver enzymes that indicate bile duct disease (high alkaline phosphatase (ALP)) and a positive auto-antibody – usually the antimitochondrial antibody (AMA). Occasionally other antibodies – anti-Sp100 or anti-gp210 – can be found, which are also diagnostic for PBC. If the autoantibodies are negative, a liver biopsy is required to establish or rule-out the diagnosis of PBC. Sometimes a liver biopsy is also done to check if you have any other liver diseases (such as autoimmune hepatitis, primary sclerosing cholangitis, fatty liver). Rarely does a liver biopsy need to be done to check the stage of your disease, as nowadays it is done by blood tests and FibroScan.
— Dr. Inbal Houri, 2023

Is there a link between diabetes and PBC?

Type 2 diabetes is a common disease in the population that is not related to PBC.
— Dr. Inbal Houri, 2023

Could there be spontaneous remission and negative AMA serology of prior positive and abnormal liver function tests (LFT) in PBC patients? If so, how often; and is it seen with Urso treatment?

Antimitochondrial antibody (AMA) positivity may often fluctuate in PBC (i.e. go from positive to negative), but that does not mean that the PBC has gone away. Currently there is no cure for PBC (to make the disease “go away” completely). It is a chronic disease that can be controlled by treatment with ursodeoxycholic acid (UDCA or Urso) – which needs to be taken as a lifelong treatment.
— Dr. Inbal Houri, 2023

After a biopsy confirming diagnosis of PBC, what criteria is used for assessing the need for a repeat biopsy?

A repeat biopsy is not often needed with PBC, unless there is a concern that the person has developed another liver disease in addition to the PBC. This would be when a person’s liver enzymes increase unexpectedly, or the liver scarring or function gets worse despite a normal or stable ALP. The most common liver disease that occurs with PBC is non-alcoholic fatty liver disease (otherwise known as “fatty liver”). Another common liver disease that can occur with PBC is an autoimmune liver disease called “autoimmune hepatitis”. Lastly, if there is concern that you may have cancer in your liver (which rarely occurs in people with cirrhosis due to PBC), you may need a liver biopsy.
— Dr. Inbal Houri, 2023

What research is currently being done with PBC?

The main clinical trials studying second line therapy for PBC have focused on medications called “PPAR” inhibitors. They work in much the same way as obeticholic acid (OCA, or Ocaliva) and ursodeoxycholic acid (UDCA or Urso) by increasing bile flow and reducing liver tests. The interest in PPAR inhibitors also focuses on their possible ability to impact on itching and improved sleep. We do not know yet whether they will truly impact on fatigue, which remains an unsolved problem. My group has an interest in studying how infections may trigger fatigue and have been looking at changes in mitochondrial function in patients with PBC. We have found that these mitochondrial and metabolic changes may be triggered by a betaretrovirus that we characterized in PBC patients. At the University of Alberta, we completed a randomized controlled trial looking at how combination anti-viral treatment with Truvada and Kaletra impact on patients with PBC. While two thirds of the patients could not tolerate the Kaletra, we found that some patients on antiviral therapy experienced improvement in their liver tests, liver biopsy and had some symptomatic improvement. So we are still looking for other antiretroviral medications with a better side effect profile that will be both well-tolerated and effective in halting disease.
—Dr. Andrew Mason, 2023

PBC Healthy Diet and Exercise

Have there been any studies on the relationship between nutrition and liver health (in order to maximize liver function in the PBC patient)?

In general, PBC patients should follow a healthy, well-balanced diet. If a patient is from European descent, then testing for celiac disease would be reasonable (will be present in roughly 6% of these PBC patients). If a patient is overweight, they may have some fat infiltration in the liver which can be associated with liver damage. In this case, losing weight through a well-balanced diet (reduced carbohydrate and fast food intake, stopping fructose containing beverages like pop) and an exercise program to maintain a normal or near normal body mass index (BMI), would be reasonable.
—Dr. Mark G. Swain, 2023

Is it bad for the liver to go for extended periods without food?

It depends upon how long of a period one is going without food. In general, extended periods of food deprivation will not hurt your liver.
—Dr. Mark G. Swain, 2023

Would PBC patients benefit from a high protein diet?

A high-protein diet is recommended for patients with cirrhosis to prevent loss of muscle mass. This can be taken through food or dietary supplements like Ensure or Boost. PBC patients without cirrhosis should take regular amounts of protein in their diet.
— Dr. Inbal Houri, 2023

Is there a recommended diet to help someone struggling to lose weight?

There is no specific diet recommended for PBC patients trying to lose weight. The general recommendations are for a well-balanced diet and exercise. The Mediterranean diet is considered liver-healthy, but there are different options and should be personalized based on preferences and routine. For exercise, the recommendation is for aerobic activity for at least 30 minutes three to four times a week (e.g. walking, swimming). The most important tip is to remember these are lifestyle changes and not short-term programs, so incorporating changes into the daily routine in a sustainable way is the best long-term plan.
— Dr. Inbal Houri, 2023