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                                  Autoimmune primary biliary cirrhosis

     

              Many liver syndromes are autoimmune and easily and permanently treatable please read our e-book for permanent cures.

 In primary biliary cirrhosis (PBC) the bile ducts in the liver are attacked by the bodys own immune system.
Bile ducts are tiny tubes in the liver that remove  bile and send it to the gallbladder, . Bile usually works to break up the fat in food into small droplets, and helps the body to absorb vitamins A, D, E and K from our diet, so it plays an important part in digestion.
When the bile ducts are attacked, they are damaged and swell, and cannot carry out these functions properly. It also means that toxic (poisonous) substances, which are normally excreted from the body through the bile, are instead kept inside the liver. These substances, such as bile acids and minerals, lead to further damage to the bile ducts and the liver tissue.
The liver can normally heal itself without scarring, but if the damage is continuous and severe, the regrowth process goes wrong and scarring occurs, meaning the liver cannot function properly. This severe damage is known as cirrhosis.
Despite its name, not everybody with primary biliary cirrhosis actually progresses to cirrhosis, because this condition often happens very slowly and with no symptoms for years.
Cirrhosis is not always, as is often assumed, related to the over-consumption of alcohol. It often has quite different causes such as inherited liver diseases. However, those with PBC can often not tolerate alcohol because their liver no longer processes it properly.
Primary biliary cirrhosis is a cholestatic condition (tending to diminish or stop the flow of bile). Because the bile can no longer break down fatty substances fully, it is frequently associated with high levels of cholesterol in the blood, which can be a risk factor for heart disease, and may lead to liver failure. Because the liver cannot carry out its usual functions, there are many other potential complications such as osteoporosis.
Women are nine times more likely than men to have this condition, and it most commonly appears between the ages of 30 and 60.

Symptoms of primary biliary cirrhosis include:
Extreme tiredness, Itchiness (pruritus), Lack of appetite. Nausea, Joint or bone pain. Arthritis. Pain over the liver (the upper right hand side below the ribs).

Skin changes such as blotchiness, or jaundice (when the skin and the whites of the eyes turn a yellowish colour). Dryness in the mouth or eyes . Diarrhea, Sometimes there are no symptoms, especially if it is mild PBC may be discovered accidentally, for example during a routine blood test.

 Cause:  The immune system starts to attack the bile ducts , causing a autoimmune diseases. More common in people who are alcoholics.

 Diagnosis : When symptoms of primary biliary cirrhosis are presented to a doctor, the first step in diagnosis is often a blood test for a substance called AMA (Anti-mitochondrial Antibody). This antibody is a substance produced by the disease-fighting system of the body and around 95% of those with PBC will test positive for this antibody.
In the early stages of PBC the liver is smooth and enlarged. As the condition progresses, nodules (bumps) appear and the liver becomes stained with bile.
Blood tests to check liver function, and a liver biopsy (a test on a tiny sample of tissue), help to determine levels of waste substances such as copper, or bodily substances such as bilirubin. If levels were particularly high, it would point to the condition.
X-rays and ultrasound scans can also be used to determine the size of the liver and rule out other causes
 

Treatment: There is no complete cure for primary biliary cirrhosis but the following therapies can have some effect in managing the condition:
Ursodeoxycholic acid has been shown to have some effect in improving the passage of bile throughout the liver, and seems to have few side effects. Immunosuppressive therapy involves using drugs such as Cyclosporine that interfere with immune reactions in the body. Liver transplantation may be considered in response to liver failure, or if the patient has uncontrollable pruritus or severe osteoporosis. Symptoms can be alleviated through various types of therapies:
Corticosteroids are drugs that may alleviate symptoms but there is a risk of osteoporosis developing if they are used long term. Antihistamines are sometimes used but mainly for sedative effect. Pruritus can be treated with drugs such as cholestyramine or therapies including ultraviolet light exposure. Treatment of sicca syndrome (dryness of eyes or mouth) can be carried out using substances such as artificial tears, lubricating jelly and oestrogen creams, depending on the symptoms. Other useful measures include:
Vitamin replacement;Checking thyroid activity;Weight-bearing exercise and calcium supplements to reduce likelihood of osteoporosis.

 

 

www.cidpusa.org  www.cidpusa.org/P/ivig.htm  http://www.cidpusa.org/disease.html http://www.cidpusa.org/Lahore.html 

  Adopted from the NHS in UK