Primary Biliary Cirrhosis

Definition

Definition of 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, 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).

Symptoms

Symptoms of Primary Biliary Cirrhosis
Although some people with primary biliary cirrhosis remain symptom-free for years after they're diagnosed, others experience a number of signs and symptoms, such as:

1. Fatigue
2. Itchy skin
3. Pain in the upper right portion of the abdomen
4. Dry eyes
5. Dry mouth
6. Yellowing of the skin and eyes (jaundice)
7. Darkening of the skin that's not related to sun exposure
8. Swollen feet and ankles
9. Accumulation of fluid in the abdomen (ascites)
10. Fatty deposits on the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees (xanthomas)
11. Diarrhea that can be greasy

Causes

Causes of Primary Biliary Cirrhosis
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.

Diagnosis

Diagnosis of Primary Biliary Cirrhosis
To diagnose PBC, distinctions should be established from other conditions with similar symptoms, such as autoimmune hepatitis or primary sclerosing cholangitis (PSC).

Diagnostic blood tests include:

1. Deranged liver function tests (elevated gamma-glutamyl transferase and alkaline phosphatase)
2. Presence of certain antibodies: antimitochondrial antibody (AMA), antinuclear antibody (ANA)

Treatment

Treatment of Primary Biliary Cirrhosis
Because no cure exists for primary biliary cirrhosis, treatment focuses on slowing the progress of the disease, relieving symptoms and preventing complications.

Prognosis

Prognosis of Primary Biliary Cirrhosis
The serum bilirubin level is an indicator of the prognosis of primary biliary cirrhosis, with levels of 2–6 mg/dL having a mean survival time of 4.1 years, 6–10 mg/dL having 2.1 years and those above 10 mg/dL having a mean survival time of 1.4 years.

Prevention

Prevention of Primary Biliary Cirrhosis
Consult with your doctor.