 |
 |
|
|
|
|
DESCRIPTION
Primary biliary cirrhosis (PBC) is a chronic, or long-term, autoimmune disease which affects primarily the bile ducts of the liver. PBC is characterized by inflammatory destruction of the intrahepatic biliary system. The origin of the disease still remains an enigma.
The first hint of autoimmune disorder in PBC came with the recognition that the blood of patients with the disease gave a high-titer reaction in the autoantibody test. Soon it was recognized that antimitochondrial reactivity was highly specific for the disease. Subsequently, evidence accumulated for many autoimmune disorders in PBC and for the likelihood that the disease had a multisystem expression but with the intrahepatic biliary system as the main target. The autoimmune attack causes inflammation and, eventually, damage to the bile ducts of the liver. Chronic inflammation causes scar tissue to form in the bile ducts and results in interference with the necessary excretion of bile.
PBC also may be described as chronic non-suppurative destructive cholangitis or as primary autoimmune cholangitis. Cirrhosis is actually a late manifestation of the disease. PBC ranges from very mild, when it may not even be detected, to a life-threatening illness. Early diagnosis and treatment is important to the outcome of the illness.
SIGNS & SYMPTOMS
PBC slowly destroys the medium-sized bile ducts within the liver. Bile is a digestive liquid that is made in the liver. Bile is essential for the proper digestion of fats and helps rid the body of worn-out red blood cells, cholesterol and potentially toxic metals. Bile travels through the bile ducts to the small intestine, where it helps digest fats and fatty vitamins. In patients with PBC, the bile ducts are destroyed by inflammation. This causes bile to remain in the liver causing harmful substances to build up in the liver, where gradual injury damages liver cells and causes irreversible cirrhosis, or scarring of the liver. As cirrhosis progresses and the amount of scar tissue in the liver increases, the liver loses its ability to function. Cirrhosis also prevents blood from the intestines from returning to the heart.
PBC advances slowly. Many patients lead active and productive lives for more than 10 to 15 years after diagnosis. Patients who show no symptoms at the time of diagnosis often remain symptom-free for years. Patients who have normal liver tests on treatment may have a normal life expectancy. PBC is a chronic illness and may lead to life-threatening complications, especially after cirrhosis develops.
Many people with PBC do not have symptoms, especially in the early stages of the disease. When symptoms do occur the most common is pruritus, or intense itching of the skin, often in the arms, legs and back. Other symptoms may include fatigue, jaundice (yellow skin caused by bilirubin retention), fluid build-up in the ankles and abdomen, and darkening of the skin and collection of fatty deposits in the skin around the eye. Jaundice, is an indication of how far the disease has progressed.
Several patients also present with complaints related to chronic portal hypertension (increased blood pressure in the veins that go to the liver that can lead to symptoms such as bleeding in the esophagus or fluid retention in the abdomen).
Several other disorders often occur with PBC. The most common is problems with the tear and salivary glands, causing dry eyes or mouth. Arthritis and thyroid problems may also be present. Renal stones and gallstones may develop. Bone softening and fragility leading to fractures can occur in late stages of the disease.
COMMON EARLY-STAGE PBC SYMPTOMS
Although some people with primary biliary cirrhosis remain symptom-free for years after they are diagnosed, others experience fatigue, itching, dry eyes and dry mouth early in the disease:
- Fatigue. This is the most common symptom of primary biliary cirrhosis. In general, energy levels are normal in the morning, but fall later in the day and do not improve with rest. Health care providers have not found any correlation between the degree of exhaustion and the severity of the illness. This means that people with mild primary biliary cirrhosis and those with more serious disease may be equally fatigued.
- Itching. Another common symptom, itching (pruritis), is often most bothersome over your legs, arms and back. The severity of itching may change, often becoming worse at night and improving during the day. Nighttime itching can disturb sleep, making fatigue worse and sometimes leading to depression. The cause of this severe itching is not clear. Note from MoonDragon.org: This could be a symptom related to the build-up of toxic substances in the body from a liver not functioning properly in filtering out and eliminating these toxins. The skin, along with the liver and kidneys, works at eliminating many toxic substances. This is done through sweat excretions. When the liver is not working properly, the kidneys and the skin must try to handle the extra work load. It could also be an autoimmune reaction of a stressed immune system.
- Dry Eyes & Mouth (Sicca Syndrome). Sicca syndrome often occurs in people with other autoimmune disorders. It causes inflammation in the moisture-secreting glands of the eyes and mouth, resulting in the decreased production of tears and saliva. This can lead to difficulty swallowing, light sensitivity and corneal ulcers.
COMMON LATER-STAGE PBC SYMPTOMS
As the destruction of bile duct and liver cells progresses, other signs and symptoms may develop, such as:
- Jaundice. A common sign of advanced liver disease, jaundice turns your skin and the whites of your eyes yellow. The discoloration is due to high blood levels of bilirubin, a byproduct of the breakdown of the hemoglobin from old or damaged red blood cells. Normally, bile carries bilirubin out of the liver so that it can be excreted from your body. But as more bile ducts are destroyed and the flow of bile slows, bilirubin begins to build up in the blood and eventually becomes visible in your skin and eyes.
- Hyperpigmentation. Inadequate bile flow increases the production of the skin pigment melanin. This causes your skin to become darker, even in areas that are not exposed to the sun. Sometimes the deeper color is not uniform, and your skin appears blotchy.
- Swollen Feet (Edema) & Abdomen (Ascites). As liver damage progresses, your body begins to retain salt and fluids. At first, the excess water accumulates mainly in your feet and ankles, which tend to become more swollen late in the day. In time, fluid can also collect in your abdomen.
- Cholesterol Deposits (Xanthomas). Your body uses bile as the main way of eliminating excess cholesterol. When disease interferes with this process, the amount of cholesterol in the blood increases. This can lead to the formation of fatty deposits in the skin around the eyes, the eyelids, or in the creases in the palms, soles, elbows, knees or buttocks. These raised, waxy growths usually do not appear until blood cholesterol reaches very high levels. Even then, not everyone with primary biliary cirrhosis develops them.
- Digestive Problems. Because bile is essential for the digestion and absorption of fats, primary biliary cirrhosis can cause intestinal problems. These include diarrhea and steatorrhea (greasy, bad-smelling stools that result from poor fat digestion).
- Urinary Tract Infections. About one in five women with primary biliary cirrhosis experiences recurring urinary tract infections. The relationship between these infections and bile duct destruction is not well understood. A few studies suggest that the same bacteria responsible for urinary tract infections may play a role in some cases of primary biliary cirrhosis.
CAUSE
The cause of this disease is unknown. It may be related to problems in the immune system (since there are antibodies, it is considered to be an autoimmune disease). Although PBC is technically not considered a hereditary disease at this time, meaning a disease caused by a known specific gene or genetic defect that is passed from parent to child, there appears to be some family link. PBC is more common among siblings and in families where one member has been affected. Environmental factors may play a part in the cause of PBC.
BILE IMPORTANCE
Bile is a greenish-yellow fluid produced in the cells of the liver. It's essential for digesting and absorbing fats and is one of the main avenues for eliminating drugs, cholesterol and metabolic byproducts from the body. Within the liver, bile is excreted into canal-like spaces between the liver cells, which drain into an interconnected series of thin tubes (ducts). The initial ducts are quite small, but become progressively larger as they spread through the liver, much like the branches of a tree.
Bile leaves the liver through the common hepatic duct (the "trunk" of the biliary tree) and enters the gallbladder, a small, pear-shaped organ just below the liver, through the cystic duct. The gallbladder stores bile in a highly concentrated form until you eat, when it is released into the common bile duct. From there, bile flows to the upper part of the small intestine (duodenum), where it begins to break down the fat in food.
MoonDragon's Health & Wellness: Gallbladder Disease
BILE DUCT DAMAGE & BLOCKAGE
Most of the bile that enters the small intestine is returned to the liver for reuse. Ordinarily, nearly a quart of bile passes from the liver to the intestine every day. Primary biliary cirrhosis destroys the small ducts within the liver, slowing and sometimes blocking the normal flow of bile.
The problem begins with inflammation in the smallest ducts in the liver. In time, the inflammation spreads to and destroys nearby liver cells. As these cells are destroyed, they are replaced by scar tissue (fibrosis). Over a period of years, the combination of ongoing inflammation, scarring and toxicity from trapped bile can lead to cirrhosis. Cirrhosis involves irreversible scarring of liver tissue that makes it impossible for the liver to carry out essential functions.
PBC - AN IMMUNE SYSTEM DISORDER
Most evidence suggests that primary biliary cirrhosis is an immune system disorder. The initial inflammation begins with T lymphocytes (T cells) - white blood cells that normally recognize and help defend against bacteria and fungi. T cells invade and destroy the epithelial cells lining the small bile ducts. The T cells also produce chemicals that stimulate the epithelial cells to secrete proteins that attract more T cells, thereby creating an ongoing cycle of damage.
This is a classic autoimmune response in which the body's immune system turns against its own cells. But not everything about primary biliary cirrhosis is classic. For one thing, it rarely occurs in children, even though autoimmune disorders often begin in childhood. For another, unlike most autoimmune diseases, primary biliary cirrhosis does not usually respond to drugs that suppress the immune system. For these and other reasons, researchers suspect that other factors play a part in the disease, such as:
- Genetics. Primary biliary cirrhosis is not transmitted from parent to child and so is not considered a hereditary disease. Yet because it seems to run in families, researchers suspect that some people may inherit certain immune system defects that make them more susceptible to the disorder. Other immune system genes may play a role in disease progression.
- Infection. For decades, researchers have suspected that primary biliary cirrhosis might result from a bacterial, fungal or parasitic infection. That would explain the massing of T cells in the small bile ducts and why the disease has some anomalies that distinguish it from other autoimmune disorders. But no commonplace infections have been linked to primary biliary cirrhosis. Scientists are now focusing on retroviruses, the same type of virus that causes HIV/AIDS.
PBC DIAGNOSIS
Because many PBC patients have no symptoms, the disease is often discovered through abnormal results on routine liver blood tests. Health care providers need to do several tests to confirm a diagnosis of primary biliary cirrhosis. One test looks for the presence of antimitochondrial antibodies (AMA) in the blood. This test is positive in nearly all PBC patients. Since the widespread use of routine serum biochemical analysis, many patients present for evaluation of an elevated serum alkaline phosphate activity that was detected on laboratory examination. Ultrasound exams and a liver biopsy, in which a small sample of liver tissue is removed with a needle, may also be performed.
Several tests can help diagnose primary biliary cirrhosis, including:
- Liver Function Tests. These blood tests check the levels of enzymes that may indicate liver disease in general and bile duct injury in particular. Certain liver enzymes are elevated in most people with primary biliary cirrhosis, especially alkaline phosphatase, which is produced in the bile ducts.
- Ultrasound Imaging. This non-invasive test uses high-frequency sound waves to create precise images of structures within the body, including the bile ducts. It is sometimes used to rule out other causes of bile flow blockage, such as gallstones or tumors.
- Anti-Mitochondrial Antibodies (AMAs). Found in every cell, mitochondria are the prime energy producers of the body. Antibodies are proteins in the blood that help destroy bacteria and other harmful pathogens. Most people with primary biliary cirrhosis have anti-mitochondrial antibodies (antibodies that target enzymes in the mitochondria). These antibodies almost never occur in people who do not have primary biliary cirrhosis, even if they have other liver disorders. For that reason, a positive AMA test is considered a very reliable indicator of the disease. At the same time, a small percentage of people with primary biliary cirrhosis do not have AMAs. False-positive tests, which indicate a problem where none exists, also can occur. Because an AMA test is not entirely foolproof, health care providers usually perform a liver biopsy, which can definitively confirm the presence or absence of the disease.
- Liver Biopsy. In this test, a small sample of liver tissue (biopsy) is removed and examined in a laboratory, either to confirm the diagnosis or to determine the extent (stage) of the disease. Health care providers withdraw the tissue through a small incision using a thin needle. Health care providers may take more liver biopsies as time goes on to check the progression of the disease.
- Magnetic Resonance Elastography (MRE). This relatively new test can help your health care provider diagnose primary biliary cirrhosis and may help avoid the need for liver biopsy, which is more invasive. MRE technology works by combining traditional magnetic resonance imaging (MRI) with low-frequency sounds waves. The MRI component uses a magnetic field and radio waves to create clear and detailed cross-sectional images of your body, which show size and structure of your tissues and organs. The low-frequency sound waves then help reveal physical properties of those tissues and organs - such as tissue stiffness. Stiffness of your liver may indicate cirrhosis.
PBC TESTS - RESULTS
Patients with PBC have abnormalities in several blood tests. In essentially all patients, the serum alkaline phosphatase and gamma-glutamyltranspeptidase activities are markedly elevated (these are enzymes present in the bile ducts). Serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) activities are usually moderately elevated (these are enzymes made by hepatocytes, the predominant liver cell type). The serum bilirubin concentration is normal early in the disease and raises as the disease progresses (which causes jaundice or yellow skin). Most patients have an elevated serum cholesterol concentration which is largely contained in an abnormal lipoprotein, termed lipoprotein X, which is produced in patients with bile duct obstruction. The total gamma-globulin concentration is usually normal until late in the disease when cirrhosis develops. Almost all patients with PBC have an elevated serum IgM concentration (a type of antibody). The prothrombin time (a measure of blood clotting) and serum albumin concentration (the major protein in blood that is made in the liver) are normal until cirrhosis develops later in the course of disease.
Serum autoantibodies are of primary importance in the diagnosis of PBC. Antibodies against mitochondria are characteristic of PBC and found in about 90 percent of patients. About 50 percent of patients with PBC also have antinuclear antibodies. Antibodies against mitochondrial and nuclear proteins are found in several diseases besides PBC, but the cDNA cloning of several mitochondrial proteins has shown that antibodies against specific antigens are virtually diagnostic of PBC. The same has been shown to be true for antibodies against nuclear proteins in about 25 percent of patients. Because of the presence of specific autoantibodies, PBC is thought to be an autoimmune disease. Several laboratories around the world are actively involved in determining how the immune response relates to the bile ducts destruction characteristic of the disease.
HISTOLOGY EXAMINATION OF LIVER BIOPSY
In addition to clinical and laboratory abnormalities and the presence of specific autoantibodies, histological examination (looking at tissue under the microscope) of liver tissue is of central importance in the diagnosis of PBC. Tissue for this purpose is obtained by liver biopsy which is generally an outpatient procedure. Histologically, PBC is classified into four stages.Stage I is referred to as the florid duct lesion or non-suppurative destructive cholangitis and is characterized by mononuclear inflammatory cells surrounding a small bile duct.
In stage II, there is proliferation of small bile ductules.
Stage III is characterized by fibrosis or scarring.
Stage IV is cirrhosis. These histological stages demonstrate the progression of the disease from destruction of the intrahepatic bile duct to fibrosis and cirrhosis.
Histological features of more than one stage can be seen on one liver biopsy. Because of sampling differences, the stages can also vary in liver biopsies done at different times on the same patient. In general, however, there is a gradual progression over years from the histological features of stage I to stage IV.
The diagnosis of PBC must be based on a combination of historical, laboratory, serological and histological criteria. In general, patients are middle aged women who present with pruritus early and jaundice late. Patients that present late in the course of disease may also have signs and symptoms of cirrhosis and hepatic failure. Many patients are referred for evaluation of an isolated elevated serum alkaline phosphatase activity on laboratory testing for other purposes. Essentially all patients have elevated serum alkaline phosphatase and gamma-glutamyltranspeptidase activities. The serum IgM concentration is almost always elevated. About 90 percent of patients have autoantibodies against specific mitochondrial proteins (the E2 subunits of the oxo-acid dehydrogenase complexes). Approximately 50 percent of patients have antinuclear antibodies, sometimes against very specific proteins (nuclear pore membrane protein gp210, transcriptional activator Sp100, inner nuclear membrane protein LBR). The absence of an elevated serum IgM concentration and/or specific autoantibodies should place the diagnosis of PBC in doubt. Patients with PBC must have a consistent liver biopsy. The histological findings alone are frequently not diagnostic as the florid duct lesion is often not seen and other features, such as ductular proliferation, fibrosis and biliary cirrhosis, can be seen in other liver diseases.
THE IMPORTANCE OF EARLY SCREENING FOR PBC
In some cases, health care providers may be able to discover if a person is at risk of primary biliary cirrhosis early. A 2007 study showed that first-degree relatives of those with primary biliary cirrhosis are more likely to have antibodies in their blood that increase their risk of the disease. If you have a first-degree relative who has primary biliary cirrhosis, your health care provider can screen your blood for these antibodies. By knowing whether you have them, your health care provider can more closely monitor you and may be able to provide treatment earlier, should you develop the disease. Early treatment is important and can help slow the progression of the disease.
PBC TREATMENT
Because no cure exists for primary biliary cirrhosis, treatment focuses on slowing the progress of the disease, relieving symptoms and preventing complications.
SLOWING PROGRESSION OF PBS
Treatments aimed at slowing the progression of PBS and prolonging life include:
- Ursodeoxycholic Acid (UDCA). Patients most often take a daily dose of a drug called ursodiol. This is urseodeoxycholic acid, a naturally occurring bile acid commonly used to treat gallstones. This drug may work by affecting the body's bile acids and reducing inflammation and improving the liver's ability to function in PBC patients. Although UDCA does not cure primary biliary cirrhosis, it may extend life expectancy and may delay the need for a liver transplant if started early in the disease. It is commonly considered the first line of therapy. It is less likely to help people with advanced liver damage. In addition, it does not improve fatigue, the most common and often disabling symptom of the disorder. The recommended dose is 15 mg per kilogram of body weight, and the medication must be taken every day for life, or until a liver transplant. Patients rarely experience side effects from ursodiol, however, the most frequent side effect of UDCA is diarrhea. Other possible side effects include aches and pains and trouble swallowing.
OTHER MEDICATIONS
Sometimes other drugs are used off-label or in clinical trials to treat primary biliary cirrhosis, but many have proved to have serious side effects. For example, some studies show that the drug methotrexate, which is normally used to treat arthritis, psoriasis and some types of cancer, may make primary biliary cirrhosis worse whereas others show it to be somewhat effective.
LIVER TRANSPLANT
When treatments no longer control primary biliary cirrhosis and the liver begins to fail, a liver transplant may help prolong life. Many health care providers use a mathematical model to help determine who will benefit from a liver transplant. People with primary biliary cirrhosis who have liver transplants often do very well, although the disease may recur in the new liver.
TREATING THE SYMPTOMS OF PBC
No medication has been found to improve fatigue, but several therapies may be used to help control and relieve the intense itching that often occurs in primary biliary cirrhosis:
- Cholestyramine & Colestipol. These cholesterol-lowering drugs can provide marked relief of itching in some people, but they have several drawbacks. Cholestyramine is an unpleasant-tasting powder that must be mixed with food or liquids, and it can be severely constipating. These drugs interfere with how your body metabolizes UDCA and certain other medications, as well as hormones and some vitamins.
- Rifampin. This drug is often prescribed for people who can not tolerate the side effects of cholestyramine. Rifampin does not work for everyone, and it is slow acting, sometimes taking about a month to take effect. It also can cause serious side effects or toxic reactions.
- Opioid Antagonists. Researchers are studying this class of drugs to relieve severe itching in people with primary biliary cirrhosis. Although some of these medications seem very effective, they must be administered intravenously and may cause withdrawal symptoms.
- Liver Transplant. This may be the only option for some people with unrelieved and intolerable itching.
QUESTIONS FOR YOUR HEALTH CARE PROVIDER
Once you have been diagnosed with PBC, here are some questions you may want to ask your health care provider about PBC:
- How severe is the liver damage?
- What treatment do you recommend?
- Will the treatment slow down the progression of the disease?
- Will any medication be prescribed?
- What are the side effects of the medication?
- Should I change my diet?
- Are there any supplements you would suggest that I take?
- What can be done to relieve my symptoms?
- If cirrhosis develops, is transplantation my only option?
- What lifestyle changes may be helpful for PBC patients?
Maintaining a healthy lifestyle will help patients feel better and may relieve or prevent some of the secondary symptoms of PBC. Your health care provider may recommend:
- A reduced sodium diet.
- Drinking ample quality water.
- Taking calcium and Vitamin D supplements.
- Avoiding or reducing the consumption of alcohol.
- Reducing stress.
- Exercising, particularly walking is beneficial.
- Skin Care.
- Regular dental examinations.
- Artificial tears for dry eyes.
PBC RISKS
Sex. Women account for about 90 percent of PBC cases. Immune system disorders in general affect far more women than men, but the reason for this is not known.
Age. It is most commonly diagnosed in patients between the age of 35 and 60 years.
Family History. Primary biliary cirrhosis is not considered hereditary, but having a family member with the disease increases your risk.
PBC has a worldwide prevalence of approximately 5 in 100,000 and an annual incidence of approximately 6 in 1,000,000 people. The prevalence and incidence appear to be similar in different regions of the world.
PBC RESEARCH
PBC has been known for more than 100 years, but now health care providers are able to diagnose it very early. This means that treatment can begin before the liver is severely damaged. Scientists are continuing to study the disease to find the cause and understand its development.
In addition, investigators are exploring the use of several additional medications to lessen the symptoms and control liver damage through drug therapy trials, involving a large number of patients around the world.
COMPLICATIONS ASSOCIATED WITH PBC
As liver damage progresses, people with primary biliary cirrhosis may develop a number of serious problems, including:
- Cirrhosis. The term "primary biliary cirrhosis" is not entirely accurate because cirrhosis develops only in the last stages of the disease - often many years after diagnosis. Yet when it does occur, cirrhosis can be life-threatening because it interferes with the liver's ability to carry out essential functions. Cases of primary biliary cirrhosis are divided into four stages. The first stage - inflammation of the bile ducts - is the least serious, and stage 4 - cirrhosis - the most serious. Ongoing cirrhosis can lead to liver failure, which occurs when the liver is no longer able to function.
- 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. Under normal circumstances, the pressure in this vein is considerably lower than the pressure in the arteries. But when scar tissue blocks normal circulation through the liver, blood backs up, much like water behind a dam, leading to increased pressure within the vein. And because blood does not flow normally through the liver, hormones, drugs and other toxins are not filtered properly before entering the bloodstream.
- Enlarged veins (varices). When circulation through the portal vein is slowed or blocked, blood may back up into other veins - mainly those in your stomach and esophagus. Sometimes veins also form around your navel and at the rectum. The blood vessels are thin walled, and increased pressure in the veins can cause bleeding in the upper stomach or esophagus. This is a life-threatening emergency that requires immediate medical care.
- Liver cancer. The destruction of healthy liver tissue that occurs in cirrhosis greatly increases the risk of liver cancer, primarily hepatocellular carcinoma, which affects the hepatocytes, the main type of liver cell.
- Weak bones (osteoporosis). Liver scarring interferes with the liver's ability to process vitamin D and calcium, both of which are essential for bone growth and health. As a result, weak, brittle bones and bone loss may be complications of late-stage primary biliary cirrhosis, and your health care provider may order a bone density test to look for osteoporosis.
- Vitamin deficiencies. A lack of bile affects the absorption of fats and of the fat-soluble vitamins A, D, E and K. This sometimes leads to deficiencies of these vitamins in advanced cases of primary biliary cirrhosis.
OTHER COMPLICATIONS ASSOCIATED WITH PBC
In addition to bile duct and liver damage, people with primary biliary cirrhosis are likely to have other metabolic or immune system disorders, including:
- Thyroid disease. Thyroid problems not only are common in people with primary biliary cirrhosis, but often appear long before the bile duct damage is diagnosed.
- CREST syndrome. This immune system disorder is a subset of scleroderma, a disease that leads to thickening, tightening and hardening of connective tissue. CREST syndrome can affect many body systems, including the blood vessels and esophagus, and sometimes the digestive tract, lungs and heart. People with primary biliary cirrhosis are more likely to have some, rather than all, of the signs and symptoms of CREST.
- Raynaud's phenomenon. One of the components of CREST, this disorder is common in people with primary biliary cirrhosis. It occurs when small blood vessels (capillaries) spasm in response to cold or emotional stress, blocking the flow of blood. Although not everyone with Raynaud's experiences the same signs and symptoms during an attack, the areas of affected skin generally turn white before becoming blue, cold and numb. When circulation improves, the skin usually reddens and may throb or tingle. Attacks of Raynaud's may last from a few minutes to several hours and tend to become worse over time.
- Rheumatoid arthritis. Some people with primary biliary cirrhosis have the aching joints that typify rheumatoid arthritis, another autoimmune disorder. Other people may have markers for rheumatoid arthritis in their blood but no signs or symptoms of the disease.
PREVENTING COMPLICATIONS
Working together, you and your health care provider can help prevent some of the complications that can occur with primary biliary cirrhosis:
- Increased pressure in the portal vein (portal hypertension). Your health care provider is likely to screen for portal hypertension and enlarged veins when you're first diagnosed and every few years thereafter.
- Weak bones (osteoporosis). Although it is not certain that osteoporosis can always be prevented in cases of primary biliary cirrhosis, treatment with bisphosphonates can prevent steroid-induced osteoporosis. Bisphosphonates is a group of drugs that can inhibit bone breakdown and even increase bone mass. Taking at least 1,500 milligrams of calcium and 1,000 international units of vitamin D every day also may be of benefit. Equally important are lifestyle measures such as stopping smoking and getting regular weight-bearing exercise.
- Vitamin deficiencies. Your health care provider may recommend injections of vitamin K and water-based oral supplements of vitamins A, D and E.
- Raynaud's phenomenon. Although drug and even surgical options exist for treating Raynaud's, self-care measures are often highly successful and are usually tried first. These measures include keeping your body's core temperature elevated and protecting your hands and feet from cold, both indoors and out. Biofeedback, a technique that teaches you to control certain body responses, may help. Autogenic training, a type of progressive muscle relaxation and hypnosis also may help improve blood flow to the extremities. If these methods fail, your doctor may prescribe a vasodilator such as a long-acting calcium channel blocker, a drug that can open small blood vessels and increase circulation.
PROGNOSIS
PBC is a progressive disease that leads to cirrhosis and liver failure. The time from diagnosis to end-stage liver disease can range from a few months to 20 years depending upon when the diagnosis is first made. Several mathematical models based on clinical, laboratory and histological criteria have been devised to predict disease progression. In general, the development of portal hypertension indicates a poor prognosis. The serum bilirubin concentration is the best prognostic indicator of all laboratory values. Once the serum bilirubin concentration reaches 6 mg/dl, the average life expectancy is about 2 years. At this time, patients should be evaluated for possible liver transplantation.
Despite extensive studies, medical therapy has not been shown to have a significant impact in slowing the progression of PBC.
- Patients with PBC should take vitamins and calcium to help prevent osteoporosis (loss of bone), a common complication of this disease.
- Colchicine may play a role in inhibiting liver fibrosis and improves laboratory values but not signs or symptoms.
- D-penicillamine has been studied in several series but the results have shown it to be ineffective and possibly toxic.
- Various immunosuppressive agents have been studied in patients with PBC.
- Corticosteroids are probably not effective and may aggravate the osteoporosis commonly present in patients with PBC.
- Azathioprine (Imuran), methotrexate and cyclosporin A have been examined in several studies and are still being investigated, but these agents will not likely produce radical improvements in clinical course.
- Ursodiol (Actigall or Urso), a bile acid, has been shown to improve the laboratory and clinical parameters in patients with PBC and the results of one study suggest that it may slow the progression of the disease.
- Orthotopic liver transplantation is highly successful in patients with end-stage liver disease resulting from PBC.
LIFESTYLE & NUTRITIONAL RECOMMENDATIONS
Taking good care of your overall health may help you feel better and improve some of the symptoms of primary biliary cirrhosis.
Eat a reduced-sodium diet, because if you have cirrhosis, sodium increases your risk of tissue swelling and of accumulating fluid in your abdominal cavity.
Exercise and take calcium and vitamin D supplements to help reduce your risk of developing osteoporosis.
Herbal Remedies: Liquid Calcium & Magnesium, Almond Flavor, 16 fl. oz.
Take 1-2 Tablespoons Daily. 1 tablespoon = 1200 mg calcium/600 mg magnesium plus other trace minerals.
Herbal Remedies: Calcium Ionic Mineral Supplement, Fully Absorbable, 700 +/- ppm, 16 fl. oz.
Herbal Remedies: Calcium Complex Bone Formula, Nature's Way, 100 Caps
Herbal Remedies: Bone Calcium, Now Foods, 120 Tabs
Herbal Remedies: Calcium Gluconate Powder, Now Foods, 100% Pure, 7 oz.
Herbal Remedies: Calcium Carbonate Powder, Now Foods, 12 oz.
Herbal Remedies: Coral Calcium (Okinawa), Organic, NOW Foods, 1000 mg, 100 VCaps
Herbal Remedies: Liquid Coral Calcium With ConcenTrace, Orange Vanilla, Trace Minerals, 1000 mg, 32 fl. oz.
Herbal Remedies: Dry Vitamin A & D, Nature's Way, 15,000/400 IU, 100 Caps
Herbal Remedies: Dry Vitamin D, Nature's Way, 100% Natural, 400 IU, 100 Caps
Herbal Remedies: Vitamin D, NOW Foods, 2000 IU, 120 Softgels
Herbal Remedies: Vitamin D, NOW Foods, 1000 IU, 180 Softgels
INFORMATION ABOUT OSTEOPOROSIS, NUTRITION & RELATED INFORMATION
Avoid or reduce alcohol consumption, because it speeds the progression of liver disease.
Practice good skin care to reduce itching: Avoid scratching. Use cool, wet compresses. Try cool baking soda or oatmeal baths. Avoid irritating clothes, soaps and detergents.
Use artificial tears to help reduce dry eyes.
RELATED LINKS
Primary Biliary Cirrhosis (PBC)
Primary Biliary Cirrhosis Organization AKA PBCers
GI Information: Primary Biliary Cirrhosis
Liver Letter: Primary Biliary Cirrhosis
For a full list of available products from Mountain Rose Herbs, click on banner below:
HELPFUL PRODUCTS & FURTHER EDUCATION
Prescription for Nutritional Healing: The A-To-Z Guide To Supplements
-- by Phyllis A. Balch, James F. Balch - 2nd Edition
Prescription for Nutritional Healing: The A-To-Z Guide To Supplements
-- by Phyllis A. Balch, James F. Balch - 4th Edition
Prescription for Herbal Healing: The A-To-Z Reference To Common Disorders
-- by Phyllis A. Balch
The Complete Guide to Natural Healing
If you see a suggested Amazon product "not there" as indicated by an orange box with the Amazon logo, this only means the specific product link has been changed by Amazon.com. Use the "click here" icon on the orange box and it will bring you to Amazon.com and you can do a search for a specific product using keywords and a new list of available products and prices will show. Their product and resource links are constantly changing and being upgraded. Many times there are more than one link to a specific product. Prices will vary between product distributors so it pays to shop around and do price comparisons.
Educational materials and health products are available through Amazon.com. Use the search box provided below to search for a particular item.
HERBS, OILS, & SUPPLIES
Mountain Rose Bulk Herbs
Mountain Rose Aromatherapy Oils
Click Here To Visit Herbal Remedies Product Page
A Source For Medicinal & Ritual Herbs & Supplies
Aphrodisiacs Aromatherapy Bath & Body Care Bulk Herbs By The Pound Capsules, Tablets & Extracts Chinese Herbals Electronics Extracts First Aid Products Food Products, Mixes, Seeds, Snacks & More! Hair Care Products & Supplies Herbs & Oils, Kilo & Ton Hydroponics & Gardening Kava Products Kitchen & Housewares Mushrooms Musical Instruments Oral Care & Dental Products PC & IT - Computer Supplies & Assessories Skin Care, Cosmetics, & Gift Sets Smokes, Seeds, Cacti Spices, Salts, Sweeteners, & Seasonings Sports, Toys & Games Sweets, Candy, Chips & Snacks Stimulants Sedatives Teas & Coffees
Shaman Shop Home Page
