Normally, the liver produces a substance known as bile, which is necessary for the proper digestion and absorption of fats. The bile is transported through the intrahepatic and extrahepatic bile ducts to the gallbladder to be stored and later excreted.
There is a disease where autoantibodies are produced that adhere to the intrahepatic bile ducts known as primary biliary cholangitis, previously called primary biliary cirrhosis. These antibodies are called antimitochondrial antibodies.
When attacked and destroyed, the cells of the bile ducts cause a narrowing in the intrahepatic ducts producing obstruction and chronically generate cirrhosis and liver failure.
It is important to mention that more than 90% of patients who suffer from primary biliary cirrhosis are women between the ages of 30 and 65 years.
Symptoms of primary biliary cirrhosis
In primary biliary cirrhosis there is a progressive destruction of the cells of the bile ducts, so that in the early stages of the disease there are no symptoms. In fact, almost half of patients with primary biliary cholangitis do not show symptoms during their lives, but they do present with altered liver enzymes.
Patients who present with symptoms manifest different sensations associated with intrahepatic attack and increased pressure within the bile ducts. These are:
· Generalized weakness: it is one of the main symptoms of this disease. It is due to the depletion of cells due to acid accumulation after exercise.
· Itching or pruritus: Almost half to 70% of patients with primary biliary cirrhosis are itchy due to the accumulation of bile pigments in the skin.
· Jaundice: yellowing of the skin and mucous membranes due to the accumulation of bile pigments.
· Neurological disorders: alteration of the sleep-wake cycle (insomnia at night and drowsiness during the day), deterioration of recent memory and in severe cases, incoherent language and distal tremor are evident.
· Gastrointestinal disorders: due to the abnormal production of bile, fats are not digested properly, being eliminated in large quantities in the stool, causing diarrhea with drops of fat (called steatorrhea). This can also lead to symptoms such as abdominal pain, gas buildup, and bloating.
· Skin changes: in addition to jaundice, thick and dry skin (xerosis) or dark colored areas (hyperpigmentation) can be observed. It is estimated that 4 out of 10 patients with primary biliary cirrhosis have skin changes.
· Enlarged liver (hepatomegaly) - Many patients have an enlarged liver. When the disease is very advanced, cirrhosis occurs (formation of micronodules in the liver tissue) and is associated with an enlarged spleen (splenomegaly), presence of fluid in the peritoneal cavity (ascites) and a collateral venous network in the skin of the abdomen.
· Alteration of liver enzymes: Most patients have an elevation of one of the liver enzymes called alkaline phosphatase, which rises when there is congestion within the liver. Other alterations in blood chemistry are an increase in cholesterol and a slight increase in aminotransferases.
To make the diagnosis of primary biliary cirrhosis, the doctor must carry out a good questioning and physical examination, and will also request complementary studies that will include antimitochondrial and antinuclear antibodies, which are found positive in most cases.
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What are the causes of primary biliary cirrhosis?
It is known that primary biliary cirrhosis is an autoimmune disease. However, the exact mechanisms by which they occur are unknown. There is a hypothesis that indicates that to trigger primary biliary cirrhosis, the union between the genetic factor (predisposition to acquire the disease in people with families who have autoimmune disease) and an external factor that triggers it is necessary.
The external factors that could trigger the disease are infections by viruses, bacteria (such as Chlamydia pneumoniae and Escherichia coli) and fungi. It has also been described that vaccines and some toxic agents could play an important role in their appearance.
Risks of suffering from primary biliary cirrhosis
The main risks are related to the complications of this disease. If it is not diagnosed early and treated in a timely manner, the following conditions may occur:
· Liver cirrhosis: in terminal cases.
· Portal hypertension: Which conditions the appearance of ascites (fluid in the abdominal cavity), collateral venous network, vessel growth (splenomegaly) and esophageal varices (with risk of gastrointestinal bleeding).
· Hepatic failure: that in terminal stages there is prolonged clotting times, hypoglycemia and hepatic encephalopathy (alteration of mental functions due to accumulation of ammonia).
· Steatorrhea (presence of lipids in the stool) which leads to the excessive elimination of fat-soluble vitamins in the stool.
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