Ursodeoxycholic acid (UDCA) is the most common medicine used with the aim of slowing the progression of the disease. However, research trials indicate that, at best, it only has a modest effect of slowing the disease in some people, and may not help at all in others. However, it may ease itch. It works by changing the make-up of bile in the liver which may reduce the harmful effect of bile on the liver cells. Immunosuppressive medicines are sometimes used. For example, penicillamine, azathioprine, methotrexate, cyclosporin, and steroids. These work by suppressing the immune system. But again, there is little clear cut evidence to say how effective they are in treating PBC. They all have a risk of causing significant side-effects.
ORTHOTOPIC LIVER
TRANSPLANTATION
Liver transplantation has greatly improved survival in patients with primary biliary cirrhosis, and it is the only effective treatment for those with liver failure.The survival rates are 92 percent and 85 percent at one and five years, respectively. Most patients have no signs of liver disease after orthotopic liver transplantation, but their antimitochondrialantibody status does not change. Primary biliary cirrhosis recurs in 15 percent of patients at 3 years and in 30 percent at 10 years.
|
|
COMPLICATIONS
g Although osteoporosis is often cited as a complication of PBC, the risk of osteoporosis is no more than for other women of that age unless they have cirrhosis that is a risk irrespective of aetiology.
g Renal tubular acidosis occurs in around half of patients with PBC. Copper deposition in the renal tubules or an autoimmune phenomenon may be the mechanism.
g Around 20% develop hypothyroidism
g Hepatocellular carcinoma develops in about 6% but this represents about 4% of women with the disease and 20% of men.
g There may be malabsorption of fats with steatorrhoea and fat soluble vitamin deficiency
g Complications of cirrhosis are described in the article.
CONCLUSION
Primary biliary cirrhosis is a slow, progressive disease. Once diagnosed, treatment is directed at managing symptoms and slowing down liver damage. A great deal of research is underway aimed at discovering the cause, preventing damage to the bile ducts and liver, improving symptoms, and prolonging life. Transplantation is now a standard form of treatment for advanced disease. By working closely with the physician, there is good reason to expect a favorable long-term outlook.
Source : Internet
|
