Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile. This obstruction damages liver cells and leads to scarring called cirrhosis.
Alternative NamesPBC
Causes, incidence, and risk factorsThe cause of inflamed bile ducts in the liver is not known. The disease more commonly affects middle-aged women.
Long-term bile obstruction is believed to lead to liver cirrhosis. The disease may be associated with autoimmune disorders such as:
More than half of patients have no symptoms at the time of diagnosis. Symptoms usually come on gradually and may include:
Tests for liver dysfunction:
Tests for the disease:
Therapy aims to relieve symptoms and prevent complications.
Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream and improves the survival of patients with PBC after they have taken it for 4 years.
Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (osteomalacia).
Liver transplantbefore liver failure occurs may be successful.
Expectations (prognosis)The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who've had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.
ComplicationsProgressive cirrhosis can lead to liver failure. Complications can include:
Call your health care provider if you have:
Afdhal NH. Diseases of the gall bladder and bile ducts. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 159.
Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.
Silveira MG, Lindor KD. Treatment of primary biliary cirrhosis: therapy with choleretic and immunosuppressive agents. Clin Liver Dis. 2008;12:425-443.
Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile. This obstruction damages liver cells and leads to scarring called cirrhosis.
Alternative NamesPBC
Causes, incidence, and risk factorsThe cause of inflamed bile ducts in the liver is not known. The disease more commonly affects middle-aged women.
Long-term bile obstruction is believed to lead to liver cirrhosis. The disease may be associated with autoimmune disorders such as:
More than half of patients have no symptoms at the time of diagnosis. Symptoms usually come on gradually and may include:
Tests for liver dysfunction:
Tests for the disease:
Therapy aims to relieve symptoms and prevent complications.
Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream and improves the survival of patients with PBC after they have taken it for 4 years.
Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (osteomalacia).
Liver transplantbefore liver failure occurs may be successful.
Expectations (prognosis)The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who've had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.
ComplicationsProgressive cirrhosis can lead to liver failure. Complications can include:
Call your health care provider if you have:
Afdhal NH. Diseases of the gall bladder and bile ducts. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 159.
Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.
Silveira MG, Lindor KD. Treatment of primary biliary cirrhosis: therapy with choleretic and immunosuppressive agents. Clin Liver Dis. 2008;12:425-443.
Reviewed ByReview Date: 05/04/2010
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, CA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
Primary biliary cirrhosis is the gradual destruction of the biliary system for unknown reasons.
yes
Itching is caused by a buildup of bile in the skin and fatigue are early symptoms.
It represents 15-20% of all cirrhosis
Has many features to suggest that it is an autoimmune disease, the system that drains bile from the liver into the intestines is progressively destroyed, the liver becomes scarred, leading to cirrhosis.
Ninety percent of patients with this disease are women between the ages of 35 and 60.
Possibly - One of the symptoms is Jaundice (yellowing of the eyes and skin), due to increased bilirubin in the blood.
Blood tests strongly suggest the correct diagnosis, but a liver biopsy is needed for confirmation. It is also usually necessary to x ray the biliary system to look for other causes of obstruction.
In some patients, the disease destroys the liver in as little as five years. In others, it may lie dormant for a decade or more.
A lack of vitamin metabolism (vitamins K, D, A, E) because of the absence of bile to assist in the absorption of nutrients from the intestines.
It is a progressive disease characterized by destruction of small bile ducts
Increased levels of bilirubin in the urine may be due to: * Biliary strictures * Cirrhosis * Gallstones in the biliary tract * Hepatitis with associated biliary obstruction * Surgical trauma affecting the biliary tract * Tumors of the liver or gall bladder