Your doctor will ask you about your health history and your family's health history, and perform a physical exam.
The following tests and procedures may be used to diagnose primary biliary cholangitis.
- Cholesterol test. More than half the people with primary biliary cholangitis have extreme increases in blood fats (lipids), including total cholesterol level.
- Liver tests. These blood tests check the levels of enzymes that may signal liver disease and bile duct injury.
- Antibody tests for signs of autoimmune disease. Blood tests may be done to check for anti-mitochondrial antibodies (AMAs). These substances almost never occur in people without the disease, even if they have other liver disorders. Therefore, a positive AMA test is considered a very reliable sign of the disease. However, a small number of people with primary biliary cirrhosis don't have AMAs.
Imaging tests may not be needed. However, they may help your doctor confirm a diagnosis or rule out other conditions with similar signs and symptoms. Imaging tests looking at the liver and bile ducts may include:
- Ultrasound. Ultrasound uses high-frequency sound waves to produce images of structures inside your body.
- Magnetic resonance cholangiopancreatography (MRCP). This special magnetic resonance imaging (MRI) exam creates detailed images of your organs and bile ducts.
- Magnetic resonance elastography (MRE). MRI is combined with sound waves to create a visual map (elastogram) of internal organs. The test is used to detect hardening of your liver that might be a sign of cirrhosis.
- Endoscopic retrograde cholangiopancreatography (ERCP). The doctor passes a thin, flexible tube down your throat and injects dye into the area of your small intestine where your bile ducts empty. A tiny camera attached to the end of the tube provides a picture of your bile ducts. This test may be done with or instead of an MRCP. But, it's invasive and may cause complications. With advances in MRI, it's usually not needed for diagnosis.
If the diagnosis is still uncertain, your doctor may perform a liver biopsy. A small sample of liver tissue is removed through a small incision using a thin needle. It's examined in a laboratory, either to confirm the diagnosis or to determine the extent (stage) of the disease.
Treating the disease
There's no cure for primary biliary cholangitis, but medications are available to help slow the progression of the disease and prevent complications. Options include:
- Ursodeoxycholic acid (UDCA). This medicine, also known as ursodiol (Actigall, Urso), is commonly used first. It helps move bile through your liver. UDCA doesn't cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. It's less likely to help with itching and fatigue. Side effects may include weight gain, hair loss and diarrhea.
- Obeticholic acid (Ocaliva). This is the newest medication approved by the Food and Drug Administration for primary biliary cholangitis. Studies show that when given alone or combined with ursodiol for 12 months it can help improve liver function.
- Fibrates (Tricor). Researchers aren't exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. But, when taken with UDCA, it has reduced liver inflammation and itching in some people. More studies are needed to determine long-term benefits.
- Other medications. Many other drugs have been used or studied for treating primary biliary cholangitis with mixed results. They include methotrexate (Trexall) and colchicine (Colcrys).
- Liver transplant. When medications no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. Liver transplantation is associated with very good long-term outcomes for people with primary biliary cholangitis. However, sometimes the disease comes back several years later in the transplanted liver.
Treating the symptoms
Your doctor may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable.
Treatment for fatigue
Primary biliary cholangitis causes fatigue. But, your daily habits and other health conditions can affect how tired you feel. Researchers are investigating whether a medicine called modafinil (Provigil) may help reduce fatigue in people with primary biliary cholangitis. More research is needed.
Treatment for itching
- Antihistamines such as diphenhydramine (Benadryl, others) and loratadine (Claritin, others) are commonly used to reduce itching. They may help with sleep if itching keeps you awake.
- Cholestyramine (Questran) is a powder that must be mixed with food or liquids. Though cholestyramine works for most people, the taste is unpleasant.
- Rifampin (Rifadin, others) is an antibiotic that may stop itching. Exactly how it does this is unknown. Researchers think it may block the brain's response to itch-inducing chemicals in the blood.
- Opioid antagonists such as those containing naloxone (Bunavail, Evzio) and naltrexone (Vivitrol) may help itching related to liver disease. Like rifampin, these drugs seem to reduce the itching sensation by acting on your brain.
Treatment for dry eyes and mouth
Artificial tears and saliva substitutes, available over-the-counter or by prescription, can help ease dry eyes and mouth. Chewing gum or sucking on hard candy also can help you make more saliva and relieve dry mouth.
Lifestyle and home remedies
You may feel better if you take good care of your overall health. Here are some things you can do to improve some primary biliary cholangitis symptoms and, possibly, help prevent certain complications:
- Choose reduced-sodium foods. Opt for low-sodium foods or naturally sodium-free foods, since sodium contributes to tissue swelling and to the buildup of fluid in your abdominal cavity (ascites).
- Never eat oysters or other raw shellfish. Such seafood can carry infection-causing bacteria, which can be dangerous for people with liver disease.
- Exercise most days of the week. Exercise may reduce your risk of bone loss.
- Avoid alcohol. Your liver processes the alcohol you drink, and the added stress can cause liver damage. Generally, people with primary biliary cholangitis should abstain from alcohol.
- Check with your doctor before starting new medications or dietary supplements. Because your liver isn't working normally, you'll likely be more sensitive to the effects of over-the-counter and prescription medications, as well as some dietary supplements, so check with your doctor before taking anything new.
Coping and support
Living with a chronic liver disease with no cure can be frustrating. Fatigue alone can have a profound impact on your quality of life. Each person finds ways to cope with the stress of a chronic disease. In time, you'll find what works for you. Here are some ways to get started:
- Learn about your condition. The more you understand about primary biliary cholangitis, the more active you can be in your own care. In addition to talking with your doctor, look for information at your local library and on websites affiliated with reputable organizations such as the American Liver Foundation.
- Take time for yourself. Eating well, exercising and getting enough rest can help you feel better. Try to plan ahead for times when you may need more rest.
- Get help. If friends or family want to help, let them. Primary biliary cholangitis can be exhausting, so accept the help if someone wants to do your grocery shopping, wash a load of laundry or cook your dinner. Tell those who offer to help what you need.
- Seek support. Strong relationships can help you maintain a positive attitude. If friends or family have a hard time understanding your illness, you may find that a support group can be helpful.
Preparing for your appointment
If you have signs or symptoms that worry you, make an appointment with your doctor. If you're diagnosed with primary biliary cholangitis, you may be referred to a doctor who specializes in disorders of the digestive system (gastroenterologist) or liver diseases (hepatologist).
Because there's often a lot to cover during your appointment, it's a good idea to arrive well-prepared.
What you can do
- Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
- Write down any symptoms you're experiencing, including those that may seem unrelated to the reason for which you scheduled the appointment.
- Write down key personal information, including any major stresses or recent life changes.
- Make a list of all medications, vitamins and supplements that you're taking.
- Ask a family member or friend to come with you. Sometimes it can be difficult to remember all of the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
- Write down questions to ask your doctor.
Preparing a list of questions can help you make the most of your time with your doctor. For primary biliary cholangitis, some basic questions to ask your doctor include:
- What's the most likely cause of my symptoms?
- What kinds of tests do I need to confirm the diagnosis? Do these tests require any special preparation?
- How severe is the damage to my liver?
- What treatments do you recommend for me?
- What types of side effects can I expect from treatment?
- Are there any other treatment options?
- Do I need to change my diet?
- Are there brochures or other printed material that I can take with me? What websites do you recommend?
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions that come to mind during your appointment.
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may give you more time to discuss a concern in greater detail. Your doctor may ask:
- What symptoms have you been experiencing?
- When did you first notice them?
- Do you always have symptoms or do they come and go?
- How severe are your symptoms?
- What, if anything, makes your symptoms better or worse?
- Has anyone in your family ever been diagnosed with primary biliary cholangitis?
- Do you have any chronic health conditions?
- Do you have a history of hepatitis or other liver disease?
- Does anyone in your family have liver disease?
- How much alcohol do you drink?
- What medications are you taking?
- Do you take any herbal or natural remedies?