Cirrhosis is a liver disease that primarily affects women in middle age of life. While the cause of the disease is unknown, is an autoimmune disorder. The name of the disease is not successful, because not all people with this diagnosis are at stage of cirrhosis, as discussed below. This disease is also known as chronic non supportive cholangitis. The disease is characterized by producing cholestasis, i.e. shrink or decreased excretion of bile. It assimilates other cholestatic disease called primary sclerosing cholangitis.
The exact cause of primary biliary cirrhosis is unknown. It is believed that genetic and environmental factors that induce immune system cells or T lymphocytes to attack the intrahepatic bile ducts, gradually leading to its destruction. This accumulation of toxins in the liver, particularly bile acids, detergent effect produced by liver cell damage. This damage is followed by development of inflammation, fibrosis and eventually cirrhosis and liver failure.
It has been speculated for several infectious agents that could trigger the disease. Among those involved include Chlamydophila pnumoniae the Propionate - bacterium acnes and some retroviruses. This has led to studies using anti-retroviral drugs for the treatment of this disease. The genetic component is supported by the fact that the disease is more common among relatives of those affected. Finally, fetal microchimerism, i.e. the persistence of fetal cells in the mother after pregnancy has also been proposed as related to the development of primary biliary cirrhosis.
The ideal treatment of primary biliary cirrhosis should be able to control the inflammatory and destructive process of the bile ducts and also treat symptoms resulting from chronic cholestasis. Primary biliary cirrhosis may have no symptoms for long periods of time and be researched only by blood tests. The manifestations of primary biliary cirrhosis include:
- Fatigue: One of the most common symptoms of this disease.
- Pruritus (itching): This symptom can be very intense and it is not uncommon for patients have been evaluated by several dermatologists before getting a diagnosis.
- Pigmentation of the skin: The skin may turn a darker shade in some patients.
- Jaundice: At later stages may have increased levels of bilirubin in the blood that is manifested by the characteristic yellowing of the skin and sclera (white of the eye).
- Joint pain
- Sjogren's Syndrome: Characterized by lack of tears or saliva production, causing dry mouth and conjunctival irritation.
- CREST syndrome: A form of localized scleroderma characterized inter alia by esophageal motility disorders and Raynaud's phenomenon (discoloration of hands by exposing them to cold).
- Other autoimmune diseases: Hypothyroidism is often up to 20% of patients. Other diseases such as celiac disease and ulcerative colitis are more common in those affected.
- Abdominal pain approximately 15% of patients has pain in liver area, without clear explanation.
- Osteoporosis: Affected patients are at risk for osteoporosis, i.e., decreased bone density, with the consequent risk of fractures.
- Hypercholesterolemia: Finding common. Fortunately, people with primary biliary cirrhosis do not have a higher risk of developing complications (atherosclerosis) due to high cholesterol.
Primary biliary cirrhosis has a highly variable evolution. Often tends to progress slowly but progressively variable periods of time, usually years. The treatment can slow this development, but not always able to stop or reverse the damage.
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