Primary biliary cirrhosis
Primary biliary cirrhosis
Primary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly destroyed. Bile, a fluid produced in your liver, is essential for the proper digestion of fats. It also helps rid your body of worn-out red blood cells, cholesterol and toxins. In primary biliary cirrhosis, the destruction of your bile ducts can cause harmful substances to build up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).
The cause of primary biliary cirrhosis remains unclear. Many experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells, although it's likely that genetic and environmental factors also play a part. Primary biliary cirrhosis develops slowly. Medication can slow the progression of the disease, especially if treatment begins early.
The exact cause of primary biliary cirrhosis isn't known, but it appears to be an immune system disorder that slowly destroys the bile ducts in your liver. Genetics and the environment also likely play a role in this disease.
Normally, bile is excreted into canal-like spaces between your liver cells, which drain into an interconnected series of thin tubes (ducts). The initial ducts are quite small, but become progressively larger as they spread through your liver, much like the branches of a tree.
Origin of the condition
The inflammation begins when T lymphocytes (T cells) begin accumulating in your liver. T cells are white blood cells that are part of your immune system response. Normally, T cells recognize and help defend against bacteria and fungi. But in primary biliary cirrhosis, the T cells invade and destroy the cells lining the small bile ducts. The T cells also produce chemicals that stimulate liver cells to secrete proteins that attract more T cells, thereby creating an ongoing cycle of damage.
Researchers suspect that a genetic susceptibility coupled with an environmental trigger, such as infection, may be at the root of this abnormal immune response:
Bile duct damage
The bile ducts carry bile from your liver to your small intestine. When bile ducts become damaged, bile can back up into the liver, causing damage to liver cells. This damage can lead to liver ...
The following factors may increase your risk of primary biliary cirrhosis:
As liver damage progresses, people with primary biliary cirrhosis may develop a number of serious problems, including:
Preparing for your appointment
You're likely to start by first seeing your family doctor or a general practitioner. However, you may then be referred to a doctor who specializes in disorders of the digestive system (gastroenterologist).
Because there's often a lot of ground to cover during your appointment, it's a good idea to arrive well prepared. Here's some information to help you get ready for your appointment, and what to expect from your doctor.
What you can do
Preparing a list of questions can help you make the most of your time with your doctor. For primary biliary cirrhosis, some basic questions to ask your doctor include:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.
What to expect from your doctor
Tests and diagnosis
Many people with primary biliary cirrhosis have no symptoms of the disease when they're initially diagnosed. Instead, doctors often become aware of a problem during routine blood tests or an evaluation for another condition.
If your doctor suspects primary biliary cirrhosis, several tests can help make the diagnosis, including:
Treatments and drugs
Because no cure exists for primary biliary cirrhosis, treatment focuses on slowing the progress of the disease, relieving symptoms and preventing complications.
Treating the disease
Treating the symptoms
Several therapies may be used to help control the intense itching that often occurs in primary biliary cirrhosis, including:
Lifestyle and home remedies
Taking good care of your overall health may help you feel better and improve some of the symptoms of primary biliary cirrhosis:
Coping and support
Although the prognosis for people with primary biliary cirrhosis is far better than it was in the past, it can still be difficult and frustrating to live with a chronic liver disease. When symptoms of the disease develop, such as fatigue and itching, they can seriously affect your quality of life.
Following are some strategies that may make dealing with primary biliary cirrhosis easier:
Last Updated: 2009-10-20
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