What is primary biliary cirrhosis?
Primary biliary cirrhosis (PBC) is a chronic progressive disease of the liver caused by a progressive destruction of the biliary ducts. While CBP can lead to a hepatic cirrhosis, not all patients develop it. Its cause is currently unknown. It acts as a reaction of the immune system against the liver, perhaps produced by external agents in predisposed individuals. The lesion arises around the intrahepatic biliary ducts, which causes an obstruction to the flow of bile. It tends to present in individuals between the age of 40 and 60, and 90% of the cases present in women.
What are the symptoms?
There are three phases.
-The pre-clinical phase occurs when symptoms are not present or alterations absent from analysis of liver function. The majority of patients (60%) are asymptomatic at the moment of diagnosis.
-The asymptomatic phase is characterized by alterations in liver function that are suggestive of cholestasis, or delay in flow of bile from the liver. Most significant is the increase in alkaline phosphatase (AP) in blood analysis. The length of this phase varies. Some patients remain asymptomatic indefinitely while others develop symptoms after 2 to 20 years.
-The symptomatic phase is progressive, but at varying pace. Initial symptoms include asthenia (weakness) and itchiness on the body. It often presents at night and generally it is localized in the palms of the hands and soles of feet until it appears over the entire body. Jaundice and coluria, yellow coloration of the skin and urine, respectively, often appear in advanced stages of the disease and do not fade with time. Other possible symptoms include weight loss, abdominal discomfort, xanthomas (pale spots around the eyes), and manifestations due to liposoluble vitamins. The terminal phase is characterized by decompensated cirrhosis and the accompanying complications: edemas, digestive hemorrhage tue to esophageal varices, and progressive liver failure.
How is it diagnosed?
There are multiple methods used for its diagnosis.
Physical examination may reveal skin alterations afore mentioned, as well as increased liver size.
Laboratory tests will show an increase in fats or lipids (cholesterol and triglycerides) and liver function tests. In spite of this, there has been no increase of cardiovascular disease associated with PBC. Bilirubin and transaminases remain normal. There is an increase of IgM immunoglobulin and anti-mitochondrial antibodies (AMA) which do not vary with the gravity or progression of the disease.
A hepatic biopsy is used to confirm the diagnosis, in which lesions of the biliary ducts can be observed at any point in the progression of the disease. In case of a biopsy that is only partially useful for detection, the diagnosis is achieved through collective evidence based on physical exam, lab work, and imaging.
CBP is diagnosed when at least two of the following three criteria are met:
- Detection of AMA superior to 1:40.
- Increase of AP to 2-10 fold the normal value for longer than 6 months.
- Compatible histology
Endoscopic Retrograde Cholangiopancreatography (ERCP) is a procedure used to diagnose and treat problems affecting the liver, gallbladder, biliary ducts, and the pancreas. The procedure combines x-rays with the use of an endoscope to examine the interior of the gastrointestinal system, and detect abnormalities.
During ERCP, PBC is characterized by the presence of ‘beads’ as in the following image.
What is the treatment?
It begins with general measures: normal activity, low-fat diet with appropriate intake of calories, vitamins, calcium, and proteins. Intake of alcohol must be avoided, as well as unnecessary medications. The patient will be prescribed with medications aimed at reducing itchiness and the inflammation of the bile ducts. Since the cause of PBC is unknown, a curative treatment is not currently available. However, it has been shown that medical treatment is safe, with no toxic effects, and improves the outcome of liver function. Not every patient has a complete recovery and disease continues with its progress. A liver transplant is the only option available in the late stage of the disease