How primary biliary cirrhosis develops
Inflammation in primary biliary cirrhosis begins with the accumulation of T lymphocytes (T cells) in the liver; T cells and leukocytes are involved in the body’s immune response. Normally, T cells recognize and help to defend against the invasion of harmful substances, such as bacteria; however, in primary biliary cirrhosis, T cells invade and destroy the lining cells of the small intrahepatic bile ducts.
Inflammation spreads from the smallest bile ducts and eventually damages the surrounding hepatocytes. These cells are damaged and scar tissue (fibrosis) is formed, leading to cirrhosis. Cirrhosis is scarring of the liver tissue and can make it difficult for the liver to perform its own basic functions.
The risk factors for primary biliary cirrhosis are as follows.
1. Gender. Most patients with primary biliary cirrhosis are women.
2.Age. Primary biliary cirrhosis occurs most often between the ages of 30 and 60.
3. Genetic factors. If you have a family member with the disease, you are at an increased risk of developing it.
Researchers believe that primary biliary cirrhosis is the result of a combination of genetic and environmental factors. Environmental factors include
4. infections. Researchers suspect that primary biliary cirrhosis can be triggered by bacterial, fungal or parasitic infections
5. Smoking. Smoking is associated with an increased risk of developing primary biliary cirrhosis.
6. Environmental toxins. Some studies suggest that toxic chemicals may play a role in the development of primary biliary cirrhosis.
As liver injury progresses, patients with primary biliary cirrhosis may develop a range of serious problems, including
7. cirrhosis of the liver. Cirrhosis, or scarring of liver tissue, causes the liver to have difficulty maintaining its own basic functions. Cirrhosis may occur in the later stages of primary biliary cirrhosis. Continued progression of cirrhosis may lead to liver failure, where the liver can no longer function properly.
8. Elevated portal venous pressure (portal hypertension). The venous vessels of the intestines, spleen and pancreas merge into a large vessel that enters the liver; this large vessel is called the portal vein. When cirrhotic scar tissue interferes with the normal circulation of blood to the liver, blood builds up, similar to water behind a dam, and can cause elevated venous blood pressure. Also, because blood does not flow properly through the liver, drugs and other toxins in the blood cannot be filtered out properly
9. Splenomegaly (enlarged spleen). Portal hypertension can lead to an enlarged spleen and a buildup of white blood cells and platelets
10. gallstones and bile duct stones. If bile cannot flow through the bile ducts, its may harden and form stones, causing pain and infection.
11.Dilated veins (varicose veins). When blood flow in the portal vein is poor or obstructed, blood may accumulate in other veins – mainly in the veins of the stomach and esophagus. The walls of the veins are thin, and increased blood pressure in the veins may cause bleeding in the stomach or upper esophagus. Such bleeding is a life-threatening emergency and requires immediate medical attention
12. Liver cancer. Destruction of healthy liver tissue in cirrhosis can increase the risk of liver cancer.
13. Weak bone bearing capacity (osteoporosis). Patients with primary biliary cirrhosis have a higher risk of osteoporosis (weak, brittle bones) and are prone to fractures.
14. Vitamin deficiency. Biliary deficiency affects the ability of the digestive system to absorb fats and fat-soluble vitamins A, D, E and K. As primary biliary cirrhosis progresses, it can sometimes lead to deficiencies of these vitamins.
15. Decreased intelligence (hepatic encephalopathy). Memory loss, difficulty concentrating and personality changes in some patients with primary biliary cirrhosis and liver failure.
16. Increased risk of developing other diseases. In addition to bile duct and liver injury, patients with primary biliary cirrhosis are likely to have co-morbidities with other metabolic or immune system disorders, including thyroid disease, limited scleroderma (CREST syndrome), and rheumatoid arthritis.
See your family doctor or general practitioner if you develop any signs or symptoms that bother you. If you are diagnosed with primary biliary cirrhosis, you may be referred to a doctor who specializes in digestive disorders (a gastroenterologist) or liver disease (a hepatologist).