Primary Biliary Cirrhosis

Primary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly destroyed. Your body has an intricate system of ducts designed specifically to transport bile, a fluid produced in your liver. Bile is essential for the proper digestion of fats and helps rid your body of worn-out red blood cells, cholesterol and potentially toxic metals. 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.

Signs and Symptoms

Early Stage

Although some people with primary biliary cirrhosis remain symptom-free for years after they're diagnosed, others experience fatigue, itching, dry eyes and dry mouth early in the disease:

• Fatigue. This is the most common symptom of primary biliary cirrhosis. In general, energy levels are normal in the morning, but fall later in the day and don't improve with rest. Doctors haven't found any correlation between the degree of exhaustion and the severity of the illness. This means that people with mild primary biliary cirrhosis and those with more serious disease may be equally fatigued.

• Itching. Another common symptom, itching (pruritis), is often most bothersome over your legs, arms and back. The severity of itching may change, often becoming worse at night and improving during the day. Nighttime itching can disturb sleep, making fatigue worse and sometimes leading to depression. The cause of this severe itching isn't clear.

• Dry eyes and mouth (sicca syndrome). Sicca syndrome often occurs in people with other autoimmune disorders. It causes inflammation in the moisture-secreting glands of the eyes and mouth, resulting in the decreased production of tears and saliva. This can lead to difficulty swallowing, light sensitivity and corneal ulcers.

Later Stage

As the destruction of bile duct and liver cells progresses, other signs and symptoms may develop, such as:

• Jaundice. A common sign of advanced liver disease, jaundice turns your skin and the whites of your eyes yellow. The discoloration is due to high blood levels of bilirubin, a byproduct of the breakdown of the hemoglobin from old or damaged red blood cells. Normally, bile carries bilirubin out of the liver so that it can be excreted from your body. But as more bile ducts are destroyed and the flow of bile slows, bilirubin begins to build up in the blood and eventually becomes visible in your skin and eyes.

• Hyperpigmentation. Inadequate bile flow increases the production of the skin pigment melanin. This causes your skin to become darker, even in areas that aren't exposed to the sun. Sometimes the deeper color isn't uniform, and your skin appears blotchy.

• Swollen feet (edema) and abdomen (ascites). As liver damage progresses, your body begins to retain salt and fluids. At first, the excess water accumulates mainly in your feet and ankles, which tend to become more swollen late in the day. In time, fluid can also collect in your abdomen.

• Cholesterol deposits (xanthomas). Your body uses bile as the main way of eliminating excess cholesterol. When disease interferes with this process, the amount of cholesterol in the blood increases. This can lead to the formation of fatty deposits in the skin around the eyes, the eyelids, or in the creases in the palms, soles, elbows, knees or buttocks. These raised, waxy growths usually don't appear until blood cholesterol reaches very high levels. Even then, not everyone with primary biliary cirrhosis develops them.

• Digestive problems. Because bile is essential for the digestion and absorption of fats, primary biliary cirrhosis can cause intestinal problems. These include diarrhea and steatorrhea — greasy, bad-smelling stools that result from poor fat digestion.

• Urinary tract infections. About one in five women with primary biliary cirrhosis experiences recurring urinary tract infections. The relationship between these infections and bile duct destruction isn't well understood. A few studies suggest that the same bacteria responsible for urinary tract infections may play a role in some cases of primary biliary cirrhosis.

Causes 

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 may play a role in this disease.

The Importance of Bile

Bile is a greenish-yellow fluid produced in the cells of the liver. It's essential for digesting and absorbing fats and is one of the main avenues for eliminating drugs, cholesterol and metabolic byproducts from the body. Within the liver, bile is excreted into canal-like spaces between the 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 the liver, much like the branches of a tree.

Bile leaves the liver through the common hepatic duct — the "trunk" of the biliary tree — and enters the gallbladder, a small, pear-shaped organ just below the liver, through the cystic duct. The gallbladder stores bile in a highly concentrated form until you eat, when it's released into the common bile duct. From there, bile flows to the upper part of the small intestine (duodenum), where it begins to break down the fat in food.

A slow attack on your bile ducts

Most of the bile that enters the small intestine is returned to the liver for reuse. Ordinarily, nearly a quart of bile passes from the liver to the intestine every day. Primary biliary cirrhosis destroys the small ducts within the liver, slowing and sometimes blocking the normal flow of bile.

The problem begins with inflammation in the smallest ducts in the liver. In time, the inflammation spreads to and destroys nearby liver cells. As these cells are destroyed, they're replaced by scar tissue (fibrosis). Over a period of years, the combination of ongoing inflammation, scarring and toxicity from trapped bile can lead to cirrhosis. Cirrhosis involves irreversible scarring of liver tissue that makes it impossible for the liver to carry out essential functions.

An immune system disorder?

Most evidence suggests that primary biliary cirrhosis is an immune system disorder. The initial inflammation begins with T lymphocytes (T cells) — white blood cells that normally recognize and help defend against bacteria and fungi. T cells invade and destroy the epithelial cells lining the small bile ducts. The T cells also produce chemicals that stimulate the epithelial cells to secrete proteins that attract more T cells, thereby creating an ongoing cycle of damage.

This is a classic autoimmune scenario — a case in which the body's immune system turns against its own cells. But not everything about primary biliary cirrhosis is classic. For one thing, it rarely occurs in children, even though autoimmune disorders often begin in childhood. For another, unlike most autoimmune diseases, primary biliary cirrhosis doesn't usually respond to drugs that suppress the immune system. For these and other reasons, researchers suspect that other factors play a part in the disease, such as:

• Genetics. Primary biliary cirrhosis isn't transmitted from parent to child and so isn't considered a hereditary disease. Yet because it seems to run in families, researchers suspect that some people may inherit certain immune system defects that make them more susceptible to the disorder. Other immune system genes may play a role in disease progression.

• Infection. For decades, researchers have suspected that primary biliary cirrhosis might result from a bacterial, fungal or parasitic infection. That would explain the massing of T cells in the small bile ducts and why the disease has some anomalies that distinguish it from other autoimmune disorders. But no commonplace infections have been linked to primary biliary cirrhosis. Scientists are now focusing on retroviruses, the same type of virus that causes HIV/AIDS.

Screening and Diagnosis

Many people with primary biliary cirrhosis have no symptoms of the disease when they're diagnosed. Instead, doctors often become aware of a problem during routine blood tests or an evaluation for another condition.

Several tests can help diagnose primary biliary cirrhosis, including:

• Liver function tests. These blood tests check the levels of enzymes that may indicate liver disease in general and bile duct injury in particular. Certain liver enzymes are elevated in most people with primary biliary cirrhosis, especially alkaline phosphatase, which is produced in the bile ducts.

• Ultrasound imaging. This noninvasive test uses high-frequency sound waves to create precise images of structures within the body, including the bile ducts. It's sometimes used to rule out other causes of bile flow blockage, such as gallstones or tumors.

• Anti-mitochondrial antibodies (AMAs). Found in every cell, mitochondria are the prime energy producers of the body. Antibodies are proteins in the blood that help destroy bacteria and other harmful pathogens. Most people with primary biliary cirrhosis have anti-mitochondrial antibodies — antibodies that target enzymes in the mitochondria. These antibodies almost never occur in people who don't have primary biliary cirrhosis, even if they have other liver disorders. For that reason, a positive AMA test is considered a very reliable indicator of the disease. At the same time, a small percentage of people with primary biliary cirrhosis don't have AMAs. False-positive tests, which indicate a problem where none exists, also can occur. Because an AMA test isn't entirely foolproof, doctors usually perform a liver biopsy, which can definitively confirm the presence or absence of the disease.

• Liver biopsy. In this test, a small sample of liver tissue (biopsy) is removed and examined in a laboratory, either to confirm the diagnosis or to determine the extent (stage) of the disease. Doctors withdraw the tissue through a small incision using a thin needle. Doctors may take more liver biopsies as time goes on to check the progression of the disease.

• Magnetic resonance elastography (MRE). This relatively new test can help your doctor diagnose primary biliary cirrhosis and may help avoid the need for liver biopsy, which is more invasive. MRE technology works by combining traditional magnetic resonance imaging (MRI) with low-frequency sounds waves. The MRI component uses a magnetic field and radio waves to create clear and detailed cross-sectional images of your body, which show size and structure of your tissues and organs. The low-frequency sound waves then help reveal physical properties of those tissues and organs such as tissue stiffness. Stiffness of your liver may indicate cirrhosis.

Early Screening

In some cases, doctors may be able to discover if a person is at risk of primary biliary cirrhosis early. A 2007 study showed that first-degree relatives of those with primary biliary cirrhosis are more likely to have antibodies in their blood that increase their risk of the disease. If you have a first-degree relative who has primary biliary cirrhosis, your doctor can screen your blood for these antibodies. By knowing whether you have them, your doctor can more closely monitor you and may be able to provide treatment earlier, should you develop the disease. Early treatment is important and can help slow the progression of the disease.

Treatment

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