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  • Guang Chen

Primary Biliary Cholangitis (formally known as Primary Biliary Cirrhosis)

Primary Biliary Cholangitis (PBC), formerly known as primary biliary cirrhosis, is a rare chronic autoimmune disease that leads to the gradual destruction of the small bile ducts in the liver. Complications include end-stage liver disease, metabolic bone disease and nutritional deficiencies. It tends to affect more women than men.




Symptoms

In the early stages of PBC, individuals may not experience any symptoms. However, as the disease progresses, symptoms may include but not limited to:

  • Fatigue

  • Itching

  • Dry eyes and mouth

  • Swelling of the spleen

  • Yellow skin and eyes (jaundice)

  • Diarrhea, fat in stools

  • Fluid in the abdomen

  • Edema (Swelling in the feet and ankles)

  • High cholesterol

  • Weight loss

Cause

The exact cause of PBC is not clearly understood.

Diagnosis

Diagnosis and assessment of PBC involves a combination of medical history, physical examination, and laboratory tests. Additional investigations such as imaging,, bone densitometry, liver tissue elastography and liver biopsy may be required.


Treatment

Treatment goals include reducing disease progression as well as the management of symptoms and complications. Oral medications including ursodeoxycholic acid and obeticholic acid may help slow the progression of PBC. Oral medications such as cholestyramine may help reduce itch. Most patients will benefit from a balanced diet as well as multivitamins, calcium and vitamin D supplementation. For patients with cirrhosis, liver cancer and variceal screening is recommended. In severe cases, liver transplantation may be considered.


 

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