Overview

Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic disease in which the bile ducts in your liver are slowly destroyed. Bile is a fluid made in your liver. It aids with digestion and helps your body get rid of cholesterol, toxins and worn-out red blood cells.

When the bile ducts are damaged, bile can back up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).

Primary biliary cholangitis is considered an autoimmune disease, which means your body's immune system is mistakenly attacking healthy cells and tissue. Researchers think a combination of genetic and environmental factors triggers the disease. It usually develops slowly. Medication can slow liver damage, especially if treatment begins early.

Source: http://www.mayoclinic.com

Symptoms

More than half the people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons. Symptoms eventually develop over the next five to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes.

Common early symptoms include:

• Fatigue
• Itchy skin
• Dry eyes and mouth

Later signs and symptoms may include:

• Pain in the upper right abdomen
• Swelling of the spleen
• Bone, muscle or joint (musculoskeletal) pain
• Swollen feet and ankles (edema)
• Buildup of fluid in the abdomen due to liver failure (ascites)
• Fatty deposits (xanthomas) on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees
• Yellowing of the skin and eyes (jaundice)
• Darkening of the skin that's not related to sun exposure (hyperpigmentation)
• Weak and brittle bones (osteoporosis), which can lead to fractures
• High cholesterol
• Diarrhea, which may include greasy stools (steatorrhea)
• Underactive thyroid (hypothyroidism)
• Weight loss

Source: http://www.mayoclinic.com

Causes

It's not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells.

The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells (T lymphocytes) start to collect in the liver. Normally, these immune cells detect and help defend against germs, such as bacteria. But in primary biliary cholangitis, they mistakenly destroy the healthy cells lining the small bile ducts in the liver.

Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they're replaced by scar tissue (fibrosis) that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

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Diagnosis

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.

Blood tests:

• 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.

Source: http://www.mayoclinic.com

Complications

As liver damage worsens, primary biliary cholangitis can cause serious health problems, including:

• Liver scarring (cirrhosis). Cirrhosis makes it difficult for your liver to work and may lead to liver failure. It indicates the later stage of primary biliary cholangitis. People with primary biliary cholangitis and cirrhosis have a poor prognosis and higher risk of other complications.
• Increased pressure in the portal vein (portal hypertension). Blood from your intestine, spleen and pancreas enters your liver through a large blood vessel called the portal vein. When scar tissue from cirrhosis blocks normal blood flow through your liver, blood backs up. This causes increased pressure inside the vein. Also, because blood doesn't flow normally through your liver, drugs and other toxins aren't filtered properly from your bloodstream.
• Enlarged spleen (splenomegaly). Your spleen can become swollen with white blood cells and platelets because your body no longer filters toxins out of the bloodstream as it should.
• Gallstones and bile duct stones. If bile cannot flow through the bile ducts, it may harden into stones, causing pain and infection.
• Enlarged veins (varices). When blood flow through the portal vein is slowed or blocked, blood may back up into other — usually those in your stomach and esophagus. Increased pressure may cause delicate veins to break open and bleed. Bleeding in the upper stomach or esophagus is a life-threatening emergency that requires immediate medical care.
• Liver cancer. Liver scarring (cirrhosis) increases your risk of liver cancer.
• Weak bones (osteoporosis). People with primary biliary cholangitis have an increased risk of weak, brittle bones that may break more easily.
• Vitamin deficiencies. A lack of bile affects your digestive system's ability to absorb fats and the fat-soluble vitamins, A, D, E and K. Because of this, some people with advanced primary biliary 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 problems with memory and concentration.
• Increased risk of other disease. Primary biliary cholangitis is associated with metabolic or immune system disorders, including thyroid problems, limited scleroderma (CREST syndrome) and rheumatoid arthritis.

Source: http://www.mayoclinic.com

Prevention

Working together, you and your doctor can help prevent these specific complications:

• Increased pressure in the portal vein (portal hypertension). Your doctor is likely to screen and monitor you for portal hypertension and enlarged veins if you have liver disease.
• Weak bones (osteoporosis). Exercise most days of the week can help increase your bone density. If you have osteoporosis, your treatment may include calcium and vitamin D supplements.
• Vitamin deficiencies. Your doctor may recommend supplements of vitamins A, D, E and K to improve vitamin levels. Avoid taking herbs or nutritional supplements without talking to your doctor first.

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.

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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.

Source: http://www.mayoclinic.com

Risk factors

The following factors may increase your risk of primary biliary cholangitis:

• Sex. Most people with primary biliary cholangitis are women.
• Age. It's most likely to occur in people 30 to 60 years old.
• Genetic factors. You're more likely to get the condition if you have a family member who has or had it.
• Geography. It's most common in northern Europe and North America.

Researchers think that genetic factors combined with certain environmental factors trigger primary biliary cholangitis. These environmental factors may include:

• Infections caused by bacteria, fungi or parasites
• Smoking
• Toxic chemicals

Source: http://www.mayoclinic.com