What is primary biliary cirrhosis?
Primary Biliary Cirrhosis (PBC), is a disease caused by damage to the bile ducts of the liver. These small channels carry digestive fluid or bile from the liver to the small intestine.
In the intestine, the bile helps to dissolve fats and vitamins A, D, E and K. Damage to the bile ducts allows bile to form in the liver. Over time, the accumulated bile can damage the liver. It can lead to permanent scarring and cirrhosis.
People with primary biliary cirrhosis may not develop any symptoms for up to 10 years. And if a person has an early stage (stage 1 or 2) of PBC, their life expectancy is normal.
If a person with primary biliary cirrhosis (PBC) has advanced symptoms as seen in the advanced stage, the average life expectancy is about 10-15 years according to the trusted source.
However, we are all different. Some people live longer with this disease than others. New therapies are improving the outlook for people with primary biliary cirrhosis (PBC).
In those with primary biliary cirrhosis (PBC), the bile ducts become injured, then red, and eventually permanently damaged. The bile ducts are small tubes in the liver that carry bile (the substance needed to digest food) from the liver to other parts of the digestive system.
When the bile ducts do not work, bile forms in the liver. This structure causes scarring of the liver (cirrhosis). Cirrhosis makes it hard for the liver to work properly. Primary biliary cirrhosis (PBC) is progressive, which means it will only get worse over time. If left untreated, cirrhosis can lead to liver failure and even death.
What are the symptoms of primary biliary cholangitis (PBC)?
More than half of people with primary biliary cirrhosis have no detectable symptoms when they are diagnosed. The disease can be diagnosed when blood tests are done for other reasons. Symptoms eventually develop over the next five to 20 years. People with symptoms at the time of diagnosis often have poor outcomes.
Most people with primary biliary cirrhosis (PBC) have no symptoms in the early stages. Some people find out they have this condition when a doctor examines them for another problem. As the condition progresses, the affected person may initially experience symptoms that include:
- Fatigue (fatigue)
- Itchy skin (pruritus)
- Abdominal pain
- Darkening of the skin
- Small yellow or white bumps under the skin or around the eyes
- Dry eyes and mouth
- Muscle and joint pain
In the later stages of the primary biliary cirrhosis (PBC), additional features:
- Yellowing of the skin and whites of the eyes (jaundice)
- Swelling of the legs, ankles and feet (edema)
- Swelling of the abdomen from fluid build-up (ascites)
- Internal bleeding in the upper abdomen / lower esophagus from dilated veins (types)
- Weight Loss
- Dark coloured urine
What causes primary biliary cirrhosis (PBC)?
- Primary biliary cirrhosis (PBC) is an autoimmune disease. This means your immune system mistakes tissue in your liver for a foreign invader and attacks it.
- Your immune system has an army of “killer” T cells that identify and fight off harmful invaders like bacteria and viruses. In people with a primary biliary cirrhosis, these T cells mistakenly attack the liver and damage cells in the bile ducts.
- Doctors don’t know exactly what causes this immune system attack. It’s likely triggered by both genetic and environmental causes.
- You’re more likely to develop primary biliary cirrhosis if you’re female. About 90 per cent of people diagnosed with primary biliary cirrhosis (PBC) are female, according to the American Liver Foundation.
Additional risk factors include:
- Being between the ages of 30 and 60
- Having a parent or sibling with this condition
- Smoking cigarettes
- Being exposed to certain chemicals
Risk factors for primary biliary cirrhosis
The following factors can increase your risk of primary biliary cholangitis:
- Gender: Most women with primary biliary cholangitis.
- Years: It occurs in people 30 to 60 years old.
- Genetic factors: If you have or have a family member, you are more likely to have this condition.
- Geography: It is most common in northern Europe and North America.
Researchers believe that genetic factors in combination with certain environmental factors can trigger primary biliary cholangitis. These environmental factors can include:
- Infections caused by bacteria, fungi, or parasites.
- Of smoking
- Toxic chemicals
How is primary biliary cirrhosis diagnosed?
Since primary biliary cirrhosis (PBC) doesn’t cause any symptoms in the early stages, a simple blood test prescribed by your doctor can diagnose it for another reason.
A liver specialist called your primary care physician or dermatologist can diagnose primary biliary cirrhosis (PBC). The doctor will first ask about your symptoms, medical history, and family medical history. They will also do a physical exam.
Tests used to diagnose this condition:
- Blood tests to check liver enzymes and other functions of liver function.
- Antimitochondrial antibody (AMA) test to detect autoimmune diseases
- Liver biopsy, which removes a small part of the liver for examination.
Your doctor can also do imaging tests to make a diagnosis. In addition to:
- Computed tomography
- magnetic resonance
- X-rays of the bile ducts
The following tests and procedures can be used to diagnose primary biliary cholangitis.
- Cholesterol test: More than half of people with primary biliary cholangitis have a dramatic increase in blood fats (lipids), including total cholesterol levels.
- Liver tests: These blood tests measure the levels of enzymes that indicate liver disease and bile duct damage.
- Antibody tests to detect signs of autoimmune disease: Blood tests may be done to check for the presence of anti-mitochondrial antibodies (AMA). Despite other liver disorders, these substances almost never occur in people without the disease.
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.
What are the treatment options for primary biliary cirrhosis?
Although there is no cure for primary biliary cirrhosis (PBC), treatments can improve your symptoms and protect your liver from further damage.
The first treatment that doctors usually try is ursodeoxycholic acid (UDCA) or ursodiol (Actigal, Urso). Ursodiol is a bile acid, which helps move bile from the liver to the small intestine. It helps with slow liver damage, especially if you start taking it while the disease is still in its early stages.
You must take this medicine for the rest of your life. The side effects of ursodiol include weight gain, diarrhea, and hair loss. Obeticholic acid (Ocaliva) is a new drug that is a reliable source for use in people who do not tolerate or do not respond to UDCA. This medicine reduces the amount of bile in the liver by reducing bile production and helps to expel bile from the liver.
Your doctor may also prescribe medications to treat symptoms:
- For itching: Antihistamines such as diphenhydramine (benadryl), hydroxyzine (vistaril), or cholestyramine (questron)
- For dry eyes: Artificial tears.
- For dry mouth: Saliva substitutes
You should also avoid alcohol as it can further damage your liver. If you are deficient in fat-soluble vitamins, you can take supplements to replace them. Taking calcium and vitamin D can help keep your bones strong.
Some doctors prescribe immunosuppressive medications to prevent the immune system from invading the liver. These medications include methotrexate (Rheumatrex, Trexol) and colchicine (Colcris). However, they have not been specifically proven to be effective for PBC.
The American Liver Foundation states that ursodiol works in about 50 per cent of the people who take it. Otherwise, liver damage can continue. If your liver is too damaged to function properly, you may need a liver transplant. This surgery will replace your liver from the donor to healthy.
Lifestyle and home remedies
If you take good care of your overall health, you will feel better. Here are some things you can do to improve some symptoms of primary biliary cholangitis and help prevent some complications:
- Choose foods that are low in sodium: Choose foods that are low in sodium or foods that are naturally low in sodium, since sodium contributes to inflammation of the tissues and the formation of fluid in the abdominal cavity (ascites).
- Never eat raw oysters or other shellfish: These shellfish carry the bacteria that cause infection, which is dangerous for people with liver disease.
- Exercise most days of the week: Exercise reduces the risk of losing bones.
- Avoid alcohol: Your liver processes the alcohol you drink, and excessive stress can damage it. Generally, people with primary biliary cholangitis should avoid alcohol.
- Consult your doctor before starting new medications or foods: Because your liver does not function normally, you are more sensitive to the effects of prescription and over-the-counter medications, as well as certain foods, so you should consult your doctor before taking a new one.
Complications of primary biliary cirrhosis
As liver damage worsens, primary biliary cholangitis can cause serious health problems, including:
- Liver scar (cirrhosis): Cirrhosis makes your liver work harder and can lead to liver failure. It refers to the later stage of primary biliary cholangitis. People with primary biliary cholangitis and cirrhosis are at increased risk for poor prognosis and other complications.
- Increased pressure in the portal vein (portal hypertension): Blood from the intestines, spleen, and pancreas enters the liver through large blood vessels called portal veins. When scar tissue from cirrhosis blocks normal blood flow through the liver, blood backs up. This causes an increase in pressure within the vein. Also, since blood does not flow normally through your liver, medications and other toxins from the bloodstream are not properly filtered.
- Enlarged spleen (splenomegaly): Your spleen becomes inflamed with white blood cells and platelets because your body can no longer filter toxins into the bloodstream.
- Gallstones and Bile Duct Stones: If the bile cannot flow through the bile ducts, it hardens and turns into stones, causing pain and infection.
- Dilated veins (types): When blood flow through the portal vein is slowed or blocked, blood flows back to other people, usually those in the stomach and esophagus. The increased pressure can cause sensitive veins to open up and bleed. Bleeding in the upper abdomen or esophagus is a life-threatening emergency that requires immediate medical attention.
- Liver cancer: Scarring of the liver (cirrhosis) increases your risk of liver cancer.
- Weak bones (osteoporosis): People with primary biliary cholangitis are at risk for weak and brittle bones, which can break more easily.
- Vitamin deficiencies: Feeling the lack of bile in your digestive system affects the capacity of fats and fat-soluble vitamins such as A, D, E and K. As a result, some of the modern primary bladders may have low levels of these vitamins.
- Decreased mental function (hepatic encephalopathy): Some people with primary biliary cholangitis with liver failure have personality changes and memory and concentration problems.
- Increases the risk of other diseases: Primary biliary cholangitis is associated with the immune system or metabolic disorders, including thyroid problems, limited scleroderma (CREST syndrome), and rheumatoid arthritis.
Prevention of primary biliary cirrhosis
By working together, you and your doctor can help prevent these specific problems:
- Increased pressure in the portal vein (portal hypertension): If you have liver disease, your doctor may be able to test and monitor your portal hypertension and varicose veins.
- Weak bones (osteoporosis): Exercising more days a week will increase your bone density. If you have osteoporosis, your treatment may include calcium and vitamin D supplements.
- Vitamin deficiencies: Your doctor may recommend vitamin A, D, E, and K supplements to improve vitamin levels. Avoid taking herbs or nutrients without first talking to your doctor.