Description

Primary biliary cholangitis is an autoimmune disease in which the bile ducts become inflamed and slowly destroyed. It was formerly called primary biliary cirrhosis.

Bile is a liquid produced in the liver. That helps with digestion and the absorption of certain vitamins. It also helps the body absorb fats and get rid of cholesterol, toxins, and the red blood cells. Course of the inflammation in the liver can lead to inflammation of the bile ducts and damage known as cholangitis. Sometimes, this can lead to permanent scarring of the tissue of the liver, called cirrhosis. Also, it can eventually lead to liver failure.

Although it affects both sexes, primary biliary cholangitis affects mainly women. It is considered an autoimmune disease, which means that your body's immune system mistakenly attacks healthy cells and tissues. The researchers believe that a combination of genetic and environmental factors that causes the disease. It usually develops slowly. At this time, there is no cure for primary biliary cholangitis, but medicines may slow liver damage, especially if treatment is started early.

Symptoms

More than half of people with primary biliary cholangitis does not have any noticeable symptoms when it is diagnosed. The disease can be diagnosed when blood tests are done for other reasons, such as routine testing. The symptoms eventually develop in the next 5 to 20 years. Those who have symptoms at the time of diagnosis tend to have worse outcomes.

Common early symptoms include:

  • Fatigue.
  • Itching in the skin.

Later, the signs and symptoms may include:

  • Yellowing of the skin and eyes called jaundice.
  • Dryness of the eyes and the mouth.
  • Pain in the right upper abdomen.
  • Swelling of the spleen is called splenomegaly.
  • The bones, muscles or joints.
  • Swelling of feet and ankles.
  • The accumulation of fluid in the abdomen due to liver failure, called ascites.
  • The fatty deposits called xanthomas of the skin around the eyes, the eyelids, or in the creases of the palms of the hands, soles of the feet, elbows or knees.
  • The darkening of the skin that is not related to the exposure to the sun, called hyperpigmentation.
  • Weak and brittle bones, osteoporosis, which can lead to fractures.
  • High cholesterol.
  • The diarrhea that may include stool greasy, called steatorrhea.
  • Low activity of the thyroid gland, it is called hypothyroidism.
  • The loss of weight.

Causes

It is not clear what causes primary biliary cholangitis. Many experts believe that it is an autoimmune disease in which the body turns against its own cells. The researchers believe that this autoimmune response may be triggered by environmental and genetic factors.

Inflammation of the liver is seen in primary biliary cholangitis begins when certain types of white blood cells called T-cells, also known as T lymphocytes, begin to collect in the liver. Normally, these immune cells to detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, which mistakenly destroy the healthy cells that line the small bile ducts in the liver.

Inflammation in the small ducts of the differential and, finally, damage to other cells in the liver. As the cells die, they are replaced by scar tissue, also known as fibrosis, which can lead to cirrhosis. Cirrhosis is the scarring of liver tissue that makes it difficult for your liver to function properly.

Risk factors

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

  • Sex. Most people with primary biliary cholangitis are women.
  • Age. It is more likely to occur in people 30 to 60 years of age.
  • Genetics. You are more likely to get the condition if you have a family member who has or has had.
  • Geography. It is more common in people of northern European descent, but primary biliary cholangitis can affect all ethnic groups and races.

The researchers believe that genetic factors combined with certain environmental factors that trigger primary biliary cholangitis. These environmental factors may include:

  • Infections, such as a urinary tract infection.
  • Smoking cigarettes, especially for long periods of time.
  • Exposure to toxic chemicals, such as in certain work environments.

Complications

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

  • The scarring of the liver, called cirrhosis. Cirrhosis makes it difficult for your liver to function and can lead to liver failure. This means that the later stage of primary biliary cholangitis. People with primary biliary cholangitis and cirrhosis have a low medical outlook. They also have a higher risk of other complications.
  • The increased pressure in the portal vein, called portal hypertension. The blood from the intestine, the spleen and the pancreas enters the liver through a large blood vessel called the portal vein. When the scar tissue of cirrhosis blocks the normal flow of blood through your liver, the blood builds up. This causes an increase of pressure inside the vein. Also, because the blood does not flow properly through your liver, drugs and other toxins are not filtered correctly from the bloodstream.
  • The enlarged veins are called varicose veins. When the flow of blood through the portal vein is slowed or blocked, blood may back up into other veins. Normally accumulates in those in their stomach and into the esophagus. The increased pressure can cause the delicate veins rupture and bleed. Bleeding in the upper part of the stomach or the esophagus is a life-threatening emergency. It requires immediate medical attention.
  • Enlargement of the spleen is called splenomegaly. The spleen may be swollen with white blood cells, and platelets. This is because your body doesn't filter out toxins from the bloodstream as it should.
  • Gallstones and bile duct stones. If the bile can't flow through the bile ducts, it can harden into stones in the ducts. These stones can cause pain and infection.
  • Cancer of the liver. The scarring of the liver, increases the risk of liver cancer. If you have scarring of the liver, you will have the need of regular cancer screening.
  • The weakness of the bones, called osteoporosis. People with primary biliary cholangitis are at an increased risk of weakness, fragility of the bones that can break more easily.
  • Deficiencies of vitamins. Not having enough bile affects the digestive system, the capacity of absorption of fats and fat-soluble vitamins, a, D, E and K. Because of this, some people with advanced primary biliary cholangitis may have low levels of these vitamins. Low levels can result in a variety of health problems, including night blindness and bleeding disorders.
  • High cholesterol. Up to 80% of people with primary biliary cholangitis have high cholesterol.
  • The decline of mental function, called hepatic encephalopathy. Some people with advanced primary biliary cholangitis and cirrhosis have personality changes. They may also have problems with memory and concentration.
  • Increase in the risk of other diseases. Primary biliary cholangitis is associated with other disorders, including those affecting the thyroid, skin, and joints. It can also be associated with dry eyes and mouth, a condition called Sjogren's syndrome.

Diagnosis

Your healthcare provider will ask about your health history and your family's medical history, and perform a physical examination. The following tests and procedures may be used to diagnose primary biliary cholangitis.

Blood tests:

  • Liver tests. These blood tests check the levels of certain proteins that may be a sign of liver disease and bile duct injuries.
  • The antibody tests to detect signs of autoimmune disease. Blood tests may be done to check for antibodies, anti-mitochondrial, also known as Love. These substances almost never occur in people without the disease, even if they have other disorders of the liver. Therefore, a positive AMA test is considered to be very reliable sign of the disease. However, a small number of people with primary biliary cirrhosis do not have Love .
  • Cholesterol test . More than half of people with primary biliary cholangitis have extreme increases in blood fats, including the level of total cholesterol.

Imaging tests can help your health care team to confirm a diagnosis or rule out other diseases with similar signs and symptoms. Imaging tests looking for in the liver and the bile ducts may include:

  • Ultrasound. The ultrasound uses high-frequency sound waves to produce images of structures inside the body.
  • FibroScan. The use of an ultrasound, as probes, this test can detect the scarring of the liver.
  • Magnetic resonance cholangiopancreatography, also known as the CPRM. This special mri scan creates detailed images of organs and bile ducts.
  • Magnetic resonance elastography, also known as the MRE. Magnetic resonance imaging is combined with sound waves to create a visual map of the internal organs, which is called elastogram. The test is used to detect the hardening of his liver which could be a sign of cirrhosis.

If the diagnosis is still uncertain, your health care professional may perform a biopsy of the liver. A small sample of liver tissue is removed through an incision using a thin needle. Then tested in a lab, either to confirm the diagnosis or to determine the extent of the disease.

Treatment

The treatment of the disease

There is No cure for primary biliary cholangitis, but there are medications available to help slow the progression of the disease and prevent complications. The options include:

  • The ursodeoxycholic acid. This medication, also known as UDCA or ursodiol (Actigall, Urso), is commonly used in the first place. It helps move bile through the liver. UDCA is not a cure primary biliary cholangitis, but it seems to improve liver function and reduce the scarring of the liver. It is less likely to help with the itching and fatigue. Side effects may include weight gain, hair loss, and diarrhea.
  • Obeticholic acid (Ocaliva). Studies show that when obeticholic acid given alone or in combination with ursodiol 12 months, you can help improve the function of the liver and slow hepatic fibrosis. However, their use is often limited, as they can cause increased itching.
  • Fibrates (Tricor). Researchers are not sure exactly how this medication works to help relieve primary biliary cholangitis symptoms. But, when taken with UDCA has been reduced liver inflammation and itching in some people. Further studies are needed to determine the long-term benefits.
  • Budesonide. When combined with UDCA , the corticosteroid budesonide may be of potential benefit for primary biliary cholangitis. However, this medication is associated with steroid-related side effects for people with more advanced disease. More long-term trials are necessary before the budesonide can be recommended for the treatment of this condition.
  • Liver transplant. When medications do not control anything primary biliary cholangitis and liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces 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 returns after several years in the transplanted liver.

The treatment of the symptoms

Your health care team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you feel more comfortable.

The treatment for fatigue

Primary biliary cholangitis causes fatigue. But their habits of life, proper diet and exercise, and other health conditions that can affect how you are feeling tired. It is also important to be tested to exclude disease of the thyroid, as it is more common in people with primary biliary cholangitis.

The treatment for itching

  • Antihistamines are commonly used to reduce itching. They can help with sleep, if the itching is kept awake. Antihistamines may include diphenhydramine, hydroxyzine hydrochloride and loratadine.
  • Cholestyramine powder is a powder that can stop the itching. Must be mixed with food or liquids.
  • Rifampicin is an antibiotic that can stop the itching. Exactly how it does this is unknown. The researchers believe that it can block the brain's response to the itch-inducing chemicals in the blood.
  • Opioid antagonists , such as those containing naloxone and naltrexone may help the itching associated with liver disease. As rifampicin, these drugs seem to reduce the itching sensation due to their effect on the brain.
  • Sertraline is a medicine that increases the levels of serotonin in the brain, called an inhibitor of serotonin reuptake inhibitors, or SSRIS. You can help reduce the itching.

The treatment for dryness of the eyes and the mouth

Artificial tears and saliva substitutes can help to relieve the dryness of the eyes and the mouth. They can be available with or without a prescription. Chewing gum or sucking on hard candy can also help you make more saliva and relieve dryness of the mouth.

Treatment of complications

Certain complications are commonly associated with primary biliary cholangitis. Your health care team may recommend:

  • Supplements of vitamins and minerals. If your body is not absorbing vitamins or other nutrients, you may need to take vitamins a, D, E, and K. it is also possible That you need calcium, folic acid and iron supplements.
  • Medicine to lower cholesterol. If you have high levels of cholesterol in the blood, your health care team may recommend that you take a medication known as a statin to help reduce your levels.
  • Medications to treat bone loss. If you have weak or thinning of the bones, osteoporosis, you may be prescribed medications or supplements, such as calcium and vitamin D, to reduce the loss of bone mass and improve bone density. Exercise such as walking and the use of light weights of the majority of the days of the week can help increase bone density.
  • Treatment for the increase of the pressure in the portal vein, called portal hypertension. Your health care team is likely that the screen and the monitor to portal hypertension and enlargement of the veins if you have more advanced scarring of the liver disease. Fluid in the abdomen is a common side effect of portal hypertension. For mild cases of fluid in the abdomen, your health care team may recommend reducing salt in your diet. More-severe cases may require the use of medications known as diuretics, or a procedure to drain the fluid called paracentesis.

Lifestyle and home remedies

You may feel better if you take good care of your health in general. Here are some things you can do to improve some primary biliary cholangitis symptoms and, possibly, help to prevent some complications:

  • Choose reduced-sodium food. The sodium is added to the tissue inflammation and the accumulation of fluid in the abdomen. Look for low-sodium foods or naturally sodium-free food.
  • Avoid eating oysters or other raw shellfish. This seafood can carry bacteria causing the infection. The infections can be dangerous for people with liver disease.
  • Exercise most days of the week. Exercise can reduce the risk of bone loss.
  • Do not smoke. If you don't smoke, don't start. If you currently smoke, talk with a health professional about strategies to help you quit smoking.
  • Avoid the consumption of alcohol. The liver processes the alcohol that you drink. The extra stress can cause liver damage. Generally, people with primary biliary cholangitis should not drink alcohol.
  • Check with your health care team before starting new medications or dietary supplements. Because your liver is not functioning normally, it'll most likely be more sensitive to the effects of medications and some dietary supplements. Check with your health care team before taking anything new.

Coping and support

Living with a course of liver disease that has no cure can be frustrating. The fatigue alone can have a major impact on your quality of life. Each person finds a way of dealing with the stress of an ongoing disease. In time, you'll find what works for you. Here are some ways to get started:

  • Learn about your condition. The more you know about primary biliary cholangitis, the more active you can be in their own care. In addition to talking with your health care team, to search for information in the local library and on the web sites 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 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 her grocery shopping, wash a load of clothes or cook dinner. Tell those that are offered to help to what you need.
  • Seek support. Strong relationships can help you maintain a positive attitude. If friends or family have a difficult time understanding of your illness, you may find that a support group can be helpful.

Preparing for your appointment

Make an appointment with a doctor or other health care professional if you have any symptoms that worry you.

If your healthcare provider thinks you might have primary biliary cholangitis, you may be referred to a doctor who specializes in disorders of the digestive system, called a gastroenterologist. You may also be referred to a doctor who specializes in diseases of the liver, called a hepatologist.

Because appointments can be brief, it is a good idea to be prepared. Here's some information to help you prepare.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there is anything that you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing, including any that do not seem to be related 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 the medicines, vitamins, and supplements you are taking and the dose.
  • Ask a family member or friend to come with you. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write questions to ask their health care team.

Your time with your health care team is limited, so preparing a list of questions can help you make the most of their time together. A list of questions from most important to least important in case time runs out. For primary biliary cholangitis, some basic questions to ask include:

  • What is 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 serious is the damage to my liver?
  • What treatments are recommended for me?
  • I'm going to need a liver transplant?
  • What types of side effects can I expect from treatment?
  • There are other treatment options?
  • Do I have to change my diet?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

You will probably be asked a series of questions. Be prepared to respond to them can give more time to discuss a concern. You may ask:

  • What symptoms have you been experiencing?
  • When did you first notice them?
  • Do you always have symptoms, or come and go?
  • How severe are the symptoms?
  • What, in any case, it makes your symptoms better or worse?
  • Anyone in your family ever been diagnosed with primary biliary cholangitis?
  • Do you have any ongoing health conditions?
  • Do you have a history of hepatitis or other liver disease?
  • Does anyone in your family has a disease of the liver?
  • How much alcohol do you drink?
  • What medications are you taking?
  • Do you take any herbal or natural remedies?
Symptoms and treatment of Primary biliary cholangitis