Primary biliary cirrhosis (PBC) is a chronic and progressive disease of the liver which involves destruction of the interlobular bile ducts.
In many cases, primary biliary cirrhosis causes no symptoms. However, in certain percentage of individuals this disease is known to cause the following signs and symptoms:
Blood tests to detect the levels of liver enzymes are done. The enzyme levels would give an indication of liver disease and extent of damage to bile ducts. In addition, blood work would also reveal the presence of anti-mitochondrial antibodies. The presence of antibodies in the blood would confirm PBC. This antibody test is considered to be a very important marker for PBC; as the antibodies do not show up in the blood tests of other liver conditions except this.
Imaging studies such as MRI and CT scan of the liver would have to be carried out to look for abnormalities in the organ. Biopsy of the liver tissue is also required to confirm the presence of PBC and to determine the extent of the disease . The histopathologic findings from the biopsy may enable the pathologist to stage PBC and guide the course of treatment and predict clinical outlook .
The primary goal of treatment of PBC is to manage the symptoms and prevent the disease progression. Timely initiation of treatment would prevent the onset of complications. The following are the treatment methods employed to treat PBC:
Prognosis of the disease condition would majorly depend on how soon the diagnosis was made and treatment initiated. In many cases, patients die of liver cirrhosis as liver transplant was not done on time. Secondary conditions such as hypothyroidism and anemia can develop as a result of PBC. It has been estimated that about 25% of patients who have suffered the disease for 10 years will ultimately develop liver failure.
The following are the possible complication of PBC:
The exact cause that triggers the development of such a condition is yet to be figured out. However, scientists believe that autoimmune disease condition causes such a disorder to develop. In this, the body regards its own cells as invaders and destroys them. Another factor that is thought to play foul includes infections caused by bacteria, parasites or viral agents. In addition, exposure to toxic compounds and smoking of cigarettes is also known to cause primary biliary cirrhosis.
Primary biliary cirrhosis was thought to be a rare condition. However, recent statistics point towards the fact that about 1 in every 3000 to 4000 individuals is affected by this chronic liver disease. In addition, women are thought to be more affected by this disease than men with the women to men ratio being 9:1 due to exogenous estrogen secretions . Mortality in primary biliary cirrhosis has been declining since the 1980’s because of advances in its therapeutic management .
The process of PBC primarily begins with the accumulation of the T cells of the immune system that gradually destroy the cells of the bile ducts. Such a phenomenon occurs due to faulty response of the immune system wherein the immune cells of the body regards its own cells as invaders and tries to destroy them. Thus, primary biliary cirrhosis is considered to be an autoimmune condition.
It is also postulated that patients with PBC may harbor telomere dysfunction which may lead to chromosomal instability and cellular senescence . In addition, PBC is also known to have a strong association with other autoimmune–mediated conditions such as scleroderma, telangiectasia syndrome, esophageal motility disorder, celiac disease, sclerodactyly and Raynaud phenomenon .
Primary biliary cirrhosis (PBC) is a chronic disorder of the liver characterized by inflammation of the bile ducts. This condition gradually destroys the bile ducts of the organ thereby obstructing the flow of bile in the bloodstream. As a result, the cells of the liver get damaged leading to cirrhosis.
Bile juice produced by the bile ducts aids in the process of digestion of food and also helps the body get rid of cholesterol, toxins and worn out blood cells. Damage to these ducts ultimately interferes with food digestion and causes accumulation of toxicogenic compounds in the body leading to various secondary conditions.
Primary biliary cirrhosis abbreviated as PBC is a chronic liver disease that gradually damages the bile ducts. The bile ducts get inflamed and are destroyed in the long run. Women are more affected by this condition than men.
The exact cause of PBC is not known; but autoimmune disorders are known to play foul. In this condition, the body’s immune system is known to attack the healthy liver cells causing PBC. It can also be linked to other autoimmune disorders such as thyroid disease, Sicca syndrome, celiac disease and Raynaud’s phenomenon.
In many cases, individuals with PBC do not suffer any symptoms. However, in some it is known to cause abdominal pain, fatigue, liver enlargement, hepatitis, itching, deposition of fats under the skin and diarrhea.
Blood work is done to determine the levels of serum enzymes and antibodies. Imaging studies such as CT scan and MRI also need to be done in order to study the liver and the extent of damage. In many instances, biopsy of the liver tissue is also done to confirm diagnosis.
Ursodeoxycholic acid is given to facilitate the removal of bile from the blood. In addition, medication such as colestipol is given to reduce the itching. Liver transplant may be necessary if all the above methods to treat the condition were not successful.