Primary Biliary Cirrhosis - What It Is, Symptoms and Treatmentswhich many are unaware of. In additionPrimary Biliary Cirrhosis, sometimes called PBC, is a disease in which the bile ducts of the liver are properly destroyed. Bile, a fluid produced in your liver, plays a role in the digestion of food and helps release your body from worn red blood cells, cholesterol, and toxins. When the bile ducts are damaged, as in thePrimary Biliary Cirrhosis, harmful substances can accumulate in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).
THEPrimary Biliary Cirrhosisis considered an autoimmune disease, in which the body rotates against its own cells. Researchers think it is triggered by a combination of genetic and environmental factors. THEPrimary Biliary Cirrhosisusually develops slowly and the medication may slow its progression, especially if the treatment starts early.
Causes of Primary Biliary Cirrhosis:It is not clear what causesPrimary Biliary Cirrhosis
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T cells are white blood cells that are part of the immune system response. Usually, T cells recognize and help defend against harmful invaders, such as bacteria. But in thePrimary Biliary Cirrhosis, T cells invade and destroy the cells that line the small bile ducts in your liver.
Inflammation in the smaller ducts spreads over time and destroys nearby liver cells. As these cells are destroyed, they are replaced by scar tissue (fibrosis) that can contribute to cirrhosis. Cirrhosis is scarring of liver tissue that hinders your liver to perform essential functions..
Symptoms of Primary Biliary Cirrhosis:Although some people withPrimary Biliary Cirrhosissymptoms for years after being diagnosed, others experience various signs and symptoms. Common first symptoms include:
- Itchy skin
- Dry eyes and mouth
Later signs and symptoms may include:
- Pain in the upper right abdomen
- Bone, muscular or joint pain (musculoskeletal)
- Yellow skin and eyes (jaundice)
- Darkening of the skin that is not related to sun exposure (hyperpigmentation)
- Swollen legs and ankles (edema)
- Accumulation of fluid in the abdomen due to hepatic insufficiency (ascites)
- Greasy deposits on the skin around your eyes, your eyelids or the wrinkles on your palms, soles, elbows or knees (xanthomas)
- Weak, brittle bones (osteoporosis), which can lead to fractures
- High Cholesterol
- Diarrhea that can be greasy
Risk Factors for Primary Biliary Cirrhosis:The following factors may increase your risk ofPrimary Biliary Cirrhosis:.
- Be woman.The vast majority of people withPrimary Biliary Cirrhosisare women.
- Your age.THEPrimary Biliary Cirrhosisis more likely in people 30 to 60 years of age.
- Genetic factors.Having a family member with the disease makes it more likely that you will develop it.
Researchers think that genetic factors, when combined with environmental factors, triggerPrimary Biliary Cirrhosis. These environmental factors may include:
- Infection.Researchers suspect that thePrimary Biliary Cirrhosiscan be triggered by a bacterial, fungal or parasitic infection.
- Smoke.Smoking is associated with an increased risk ofPrimary Biliary Cirrhosis.
- Environmental toxins.Some research suggests that toxic chemicals may play aPrimary Biliary Cirrhosis.
Complications of Primary Biliary Cirrhosis:As liver damage progresses, people withPrimary Biliary Cirrhosiscan develop a number of serious problems, including:
- Cirrhosis.Cirrhosis is liver scarring that makes it difficult for the liver to function normally. Cirrhosis may occur in the later stages ofPrimary Biliary Cirrhosis. Continuous cirrhosis can lead to liver failure, which occurs when the liver is no longer able to function properly.
- Increased pressure in the portal vein (portal hypertension).Blood from your gut, spleen and pancreas enters the liver through a large blood vessel called the portal vein. When the scar tissue of cirrhosis blocks the normal circulation through your liver, the copies of the blood as well as the water behind a dam, leading to increased pressure inside the vein. And because blood does not flow normally through your liver, drugs and other toxins are not adequately filtered from the bloodstream.
- Enlarged spleen (splenomegaly).Your spleen may become enlarged with white blood cells and platelets due to portal hypertension.
- Gallstones and bile stones.If bile can not flow through the bile ducts, it can stiffen into stones, causing pain and infection.
- Enlarged veins (varicose veins).When circulation through the portal vein is slowed down or blocked, the blood may return to other veins - especially those in your stomach and esophagus. Blood vessels are thin-walled and increasing pressure in your veins can cause bleeding in the upper stomach or esophagus. This bleeding is a life-threatening emergency that requires immediate medical care.
- Liver cancer.The destruction of healthy liver tissue that occurs in cirrhosis increases your risk of liver cancer.
- Weak bones (osteoporosis).People withPrimary Biliary Cirrhosishave an increased risk of weak and brittle bones that can break more easily.
- Vitamin deficiencies.The lack of bile affects the ability of your digestive system to absorb fats and fat soluble vitamins, A, D, E, and K. This sometimes leads to deficiencies of these vitamins in advanced cases ofPrimary Biliary Cirrhosis.
- Decreased mental function (hepatic encephalopathy).Some people withPrimary Biliary Cirrhosiswith hepatic insufficiency have problems of memory, concentration and personality change.
- An increased risk of another disease.In addition to biliary damage and liver damage, people withPrimary Biliary Cirrhosisyou will probably have other metabolic or immune system disorders, including thyroid problems, limited scleroderma (CREST syndrome), and rheumatoid arthritis.
Tests and Diagnosis of Primary Bilar Cirrhosis:Your doctor will first ask you about your family's health history and health history and perform a physical examination. The following tests and procedures may be used to diagnosePrimary Biliary Cirrhosis..
- Blood tests to check liver function.Liver function tests check the levels of enzymes that can indicate liver disease in general and bile duct injury in particular.
- Blood tests to check for signs of autoimmune disease.An examination of your blood can reveal anti-mitochondrial antibodies (AMAs). These antibodies almost never occur in people who do not have the disease, even if they have other liver diseases. Therefore, a positive AMA test is considered a very reliable indicator of disease. However, a small percentage of people withPrimary Biliary Cirrhosishas no AMAs.
- Ultrasound.Ultrasound uses high-frequency sound waves to produce images of structures within your body.
- Computed tomography (CT).You may have a CT scan - a special x-ray technique that provides much more information than a standard x-ray.
- Magnetic resonance imaging (MRI).An MRI scanner uses a magnetic field and radio waves to create detailed images of organs and tissues. Unlike CT, there is no exposure to radiation with magnetic resonance imaging.
- Magnetic resonance imaging (MRI).This most recent test combines images of MRI images with sound waves to create a visual map (elastogram) of internal organs. The test is used to detect the hardening of the liver that may indicate cirrhosis, similar to how a doctor would examine (feel) your body.
- X-rays of your biliary tract.You may need a type of bile duct X-ray called endoscopic retrograde cholangiopancreatography (ERCP) in addition to or instead of an MRI. To make your bilingual channels visible on an x-ray, your doctor uses a flexible tube that has passed the throat to inject ink into the small intestine area where your biliary tract is empty. This test is invasive and may result in complications. With advancements in MRI, it is generally not necessary for diagnosis.
If the diagnosis is still uncertain, doctors can perform a liver biopsy. A small sample of liver tissue is removed and examined in the laboratory, either to confirm the diagnosis or to determine the extent (stage) of the disease. Doctors remove the tissue through a small incision using a fine needle..
Primary Biliary Cirrhosis Treatments:Since there is no cure forPrimary Biliary Cirrhosis, the treatment focuses on slowing the progress of the disease, relieving symptoms and preventing complications.
Treating the Disease:Treatments to delay disease and prolong life include:.
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- Ursodeoxycholic acid (UDCA).Also known as ursodiol (Actigall, Urso), UDCA is a bile acid that helps to move bile through the liver. UDCA does not curePrimary Biliary Cirrhosis, but can prolong life if started early in the disease and is generally considered the first line of therapy. Although the UDCA does not work for everyone withPrimary Biliary Cirrhosispeople with early-stage disease respond better. UDCA is less likely to help people with advanced liver damage. Side effects may include weight gain, hair loss and diarrhea.
- Liver transplant.When treatments no longer control thePrimary Biliary Cirrhosisand the liver starts to fail, a liver transplant can help prolong life. A liver transplant is a procedure to remove your diseased liver and replace it with a healthy liver from a donor. Most livers donated come from people who died. But in some cases, it may be possible to remove a portion of a liver from a living donor. THEPrimary Biliary Cirrhosisoften repeated in the transplanted liver, but may take several years to develop. People withPrimary Biliary Cirrhosiswho have liver transplants usually have high survival rates - 80 to 85 percent after five years, on average.
- New medicines.Researchers continue to explore other drugs for the treatment ofPrimary Biliary Cirrhosis. Immunosuppressive drugs, in particular methotrexate (Trexall, Rheumatrex) and colchicine (Colcrys), have been widely used, but their efficacy remains undetected. Numerous other drugs, including antiviral drugs, continue to be studied as well. Studies suggest that the addition of fenofibrate (Tricor, Triglide, others) or bezafibrate (Bezalip, others) may be useful for some people who do not respond to UDCA alone, but larger studies are required.
Treating Symptoms:Your doctor may recommend treatments to control the signs and symptoms ofPrimary Biliary Cirrhosisto make it more comfortable. Treatments can help control the most common symptoms..
Treatment of Fatigue:Although thePrimary Biliary Cirrhosisin itself causes fatigue, your daily habits and other medical conditions can contribute to your tiredness. By addressing these other factors, you can get relief. One drug that has shown promise in studies is modafinil (Provigil). Further research is needed to determine its role inPrimary Biliary Cirrhosis.
- Antihistamines such as diphenhydramine (Benadryl, others) and loratadine (Alavert, Claritin, others) are commonly used to reduce itching by insect bites, dry eyes, and dry mouth. On the other hand, antihistamines may help with sleep if the itching keeps you awake. Cholestyramine (Questran, Prevalite) is a powder that should be mixed with food or liquid. Although cholestyramine works for most people, the taste is unpleasant.
- Rifampin (Rifadin, Rimactane, others) is an antibacterial drug that is taken in the form of a pill. Exactly how rifampicin reduces the itching is unknown but can block the brain's response to chemicals that induce itching in its circulation.
- Opioid antagonists such as naloxone (Bunavail, Evzio) and naltrexone (Vivitrol) may help itching related to liver disease. Like rifampicin, these drugs seem to reduce the itchy feeling in your brain.
Treatment for Dry Eyes and Mouth:.
- Artificial tears and saliva substitutes are available off the counter.
- Pilocarpine (Isopto Carpine, Salagen) is a prescription drug that can be used if over-the-counter medications do not help.
- Cevimeline (Evoxac) is another prescription drug for dry eyes and mouth.
Prevention of Primary Biliary Cirrhosis:Working together, you and your doctor can help prevent some of the complications that can occur withPrimary Biliary Cirrhosis:
- Increased pressure in the portal vein (portal hypertension).Your doctor will likely exhibit portal hypertension and enlarged veins when you are diagnosed for the first time and every few years thereafter. If you are diagnosed with portal hypertension or bleeding, treatment may involve medications, such as a beta blocker or nitrate, or surgery.
- Weak bones (osteoporosis).Treatment for bone loss associated withPrimary Biliary Cirrhosisusually involves calcium and vitamin D supplements. Your doctor may also recommend that you exercise most days of the week to help increase your bone density.
- Vitamin deficiencies.Your doctor may recommend vitamin A, D, E, and K supplements to combat vitamin deficiencies. Avoid taking herbs or nutritional supplements without first talking to your doctor.
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Taking good care of your overall health can help you feel better and improve some of the symptoms ofPrimary Biliary Cirrhosis:
- Choose foods with low sodium content.Choose foods low in sodium or naturally sodium-free foods, as sodium contributes to swelling of the tissues and to the accumulation of fluid in your abdominal cavity (ascites).
- Exercise on most days of the week.Exercise can reduce your risk of bone loss.
- Avoid alcohol.Your liver processes the alcohol you drink and the additional stress can cause damage to the liver. Generally, people withPrimary Biliary Cirrhosisshould abstain from alcohol.
- Check with your doctor before starting any new medicines or dietary supplements.Because your liver is not functioning normally, you will probably be more sensitive to the effects of sale and prescribed as well as some dietary supplements, so check with your doctor before taking anything new
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