Primary biliary cirrhosis

DEFINITION

Primary biliary cirrhosis, sometimes called PBC, is a disease in which the bile ducts in your liver are slowly destroyed. Bile, a fluid produced in your liver, plays a role in digesting food and helps rid your body of worn-out red blood cells, cholesterol and toxins.

When bile ducts are damaged, as in primary biliary cirrhosis, harmful substances can build up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).

Primary biliary cirrhosis is considered an autoimmune disease, in which the body turns against its own cells. Researchers think it is triggered by a combination of genetic and environmental factors. Primary biliary cirrhosis usually develops slowly and medication can slow its progression, especially if treatment begins early.

CAUSES

It’s not clear what causes primary biliary cirrhosis. Many experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells.

How primary biliary cirrhosis develops

The inflammation of primary biliary cirrhosis begins when T lymphocytes (T cells) start 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 harmful invaders, such as bacteria. But in primary biliary cirrhosis, the T cells invade and destroy the cells lining the small bile ducts in your liver.

Inflammation in the smallest ducts spreads, in time, and destroys nearby liver cells. As these cells are destroyed, they’re replaced by scar tissue (fibrosis) that can contribute to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to carry out essential functions.

SYMPTOMS

Although some people with primary biliary cirrhosis remain symptom-free for years after they’re diagnosed, others experience a number of signs and symptoms.

Common early symptoms include:

• Fatigue
• Itchy skin
• Dry eyes and mouth

Later signs and symptoms may include:

• Pain in the upper right portion of the abdomen
• Bone, muscle or joint (musculoskeletal) pain
• Yellowing of the skin and eyes (jaundice)
• Darkening of the skin that’s not related to sun exposure (hyperpigmentation)
• Swollen feet and ankles (edema)
• Buildup of fluid in the abdomen due to liver failure (ascites)
• Fatty deposits on the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees (xanthomas)
• Weak and brittle bones (osteoporosis), which can lead to fractures
• Elevated cholesterol
• Diarrhea that can be greasy (steatorrhea)
• Underactive thyroid (hypothyroidism)