Primary biliary cirrhosis

Primary biliary cirrhosis (PBC), also known as primary biliary cholangitis, is a chronic disease of the liver that slowly destroys the bile ducts within the liver.

Signs and Symptoms

The disease is often asymptomatic in its early stages, but as it progresses, symptoms may include fatigue, itching, and jaundice. Other symptoms can include dry eyes and dry mouth, and in later stages, cirrhosis and liver failure can occur.

Causes

The exact cause of PBC is unknown, but it is believed to be a combination of genetic and environmental factors. It is considered an autoimmune disease, as the body's immune system mistakenly attacks the cells of the bile ducts.

Diagnosis

Diagnosis is typically made through blood tests, including tests for antimitochondrial antibodies (AMAs), which are present in 95% of cases. Other tests may include a liver biopsy or imaging studies such as an ultrasound or MRI.

Treatment

There is currently no cure for PBC, but treatments can help manage symptoms and slow the progression of the disease. These may include medications such as ursodeoxycholic acid (UDCA) and obeticholic acid (OCA), as well as treatments for specific symptoms such as itching and fatigue. In severe cases, a liver transplant may be necessary.

Epidemiology

PBC primarily affects middle-aged women, and is more common in Northern Europe and North America. The incidence and prevalence of the disease are increasing, possibly due to increased awareness and diagnosis.

See Also

• Liver disease
• Autoimmune disease
• Bile duct

References

Primary biliary cirrhosis Resources
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